• Abbasciano V, Trevisani L, Di Todaro F, Guglielmini C, Marzola A, Sartori S, Salemi A. Primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. An early case. Recenti Prog. Med. 97: 28-31 (2006).

• Abdel Fattah S, Abdel KhaliK K, el-Shawarby L, Abdel Fattah S, el Shimi S, Soliman M, Hassan H. Some immunological aspects of chronic liver diseases in Egyptian children. J. Egypt. Soc. Parasitol. 21: 343-355 (1991).

• Abedi MR, Hammarstrom L, Broome U, Angelin B, Smith CI, Christensson B. Reduction in serum levels of antimitochondrial (M2) antibodies following immunoglobulin therapy in severe combined immunodeficient (SCID) mice reconstituted with lymphocytes from patients with primary biliary cirrhosis (PBC). Clin. Exp. Immunol. 105: 266-273 (1996). *

• Acin-Perez R, Hoyos B, Zhao F, Vinogradov V, Fischman DA, Harris RA, Leitges M, Wongsiriroj N, Blaner WS, Manfredi G, Hammerling U. Control of oxidative phosphorylation by vitamin A illuminates a fundamental role in mitochondrial energy homoeostasis. FASEB J. Oct 7 [Epub ahead of print] (2009). *

• Adachi M, Ishii H. Role of mitochondria in alcoholic liver injury. Free Radic. Biol. Med. 32: 487-491 (2002).

• Adami P, Berrez JM, Latruffe N. Mitochondrial pyruvate dehydrogenase complex subunits as autoantigens in human primary biliary cirrhosis. Biochem. Int. 23: 429-437 (1991).

• Adams JS, Chen H, Chun R, Ren S, Wu S, Gacad M, Nguyen L, Ride J, Liu P, Modlin R, Hewison M. Substrate and enzyme trafficking as a means of regulating 1,25-dihydroxyvitamin D synthesis and action: the human innate immune response. J. Bone Miner. Res. 22 Suppl. 2: V20-V24 (2007). *

• Agarwal A, Makker K, Sharma R. Clinical relevance of oxidative stress in male factor infertility: an update. Am. J. Reprod. Immunol. 59: 2-11 (2008). *

• Agarwal K, Jones DE, Watt FE, Burt AD, Floreani A, Bassendine MF. Familial primary biliary cirrhosis and autoimmune cholangitis. Dig. Liver Dis. 34: 50-52 (2002).

• Agmon-Levin N, Shapira Y, Selmi C, Barzilai O, Ram M, Szyper-Kravitz M, Sella S, Katz BS, Youinou P, Renaudineau Y, Larida B, Invernizzi P, Gershwin ME, Shoenfeld Y. A comprehensive evaluation of serum autoantibodies in primary biliary cirrhosis. J. Autoimmun. Nov 6 [Epub ahead of print] (2009).

• Akiyama Y, Tanaka M, Takeishi M, Adachi D, Mimori A, Suzuki T. Clinical, serological and genetic study in patients with CREST syndrome. Intern. Med. 39: 451-456 (2000). *

• Al Hussami O, Godinot C, Monier JM, Trepo C, Monier JC. Demonstration of anti-mitochondria antibodies by ELISA. Comparison with the indirect immunofluorescence technique on organ sections. J. Clin. Lab. Immunol. 21: 195-199 (1986).

• al-Hussami O, Godinot C, Monier JM, Trepo C, Monier JC. Use of immunoblotting to characterize the mitochondrial antigens recognized by anti-mitochondrial autoantibodies. J. Immunol. Methods 113: 61-73 (1988).

• Al-Jabri AA, Al Belushi MS, Nsanze H. Frequency and levels of autoantibodies in healthy adult Omanis. Ann. Saudi Med. 23: 372-375 (2003).

• Alard JE, Dueymes M, Youinou P, Jamin C. Modulation of endothelial cell damages by anti-Hsp60 autoantibodies in systemic autoimmune diseases. Autoimmun. Rev. 6: 438-443 (2007).

• Alderuccio F, Toh BH, Barnett AJ, Pedersen JS. Identification and characterization of mitochondria autoantigens in progressive systemic sclerosis: identity with the 72,000 dalton autoantigen in primary biliary cirrhosis. J. Immunol. 137: 1855-1859 (1986).

• Alessandri C, Sorice M, Bombardieri M, Conigliaro P, Longo A, Garofalo T, Manganelli V, Conti F, Esposti ED, Valesini G. Anti-phospholipid reactivity against cardiolipin metabolites occurring during endothelial cell apoptosis. Arthritis Res. Ther. 8: R180 (2006). *

• Allen S, Huber J, Devendra D. Prevalence of organ-specific autoantibodies in childhood- and adult-onset type 1 diabetes. Ann. N. Y. Acad. Sci. 1150: 260-262 (2008). *

• Allina J, Hu B, Sullivan DM, Fiel MI, Thung SN, Bronk SF, Huebert RC, van de Water J, LaRusso NF, Gershwin ME, Gores GJ, Odin JA. T cell targeting and phagocytosis of apoptotic biliary epithelial cells in primary biliary cirrhosis. J. Autoimmun. 27: 232-241 (2006).

• Alric L, Thebault S, Selves J, Peron JM, Mejdoubi S, Fortenfant F, Vinel JP. Characterization of overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis according to antimitochondrial antibodies status. Gastroenterol. Clin. Biol. 31: 11-16 (2007). *

• Amakasu H, Kanno A, Abe M. Multiple myeloma occurring in early stage primary biliary cirrhosis. Tohoku J. Exp. Med. 169: 197-203 (1993).

• Amano K, Leung PS, Xu Q, Marik J, Quan C, Kurth MJ, Nantz MH, Ansari AA, Lam KS, Zeniya M, Coppel RL, Gershwin ME. Xenobiotic-induced loss of tolerance in rabbits to the mitochondrial autoantigen of primary biliary cirrhosis is reversible. J. Immunol. 172: 6444-6452 (2004). *

• Amaral JD, Sola S, Steer CJ, Rodrigues CM. Role of nuclear steroid receptors in apoptosis. Curr. Med. Chem. 16: 3886-3902 (2009).

• Ambinder EP, Cohen LB, Wolke AM, Field SP, Adelsberg B, Schaffner F, Zaroulis CG. The clinical effectiveness and safety of chronic plasmapheresis in patients with primary biliary cirrhosis. J. Clin. Apheresis 2: 219-223 (1985).

• Amezcua-Guerra LM, Prieto P, Bojalil R, Pineda C, Amigo MC. Microscopic polyangiitis associated with primary biliary cirrhosis: a causal or casual association? J. Rheumatol. 33: 2351-2353 (2006).

• Anand AC, Elias E, Neuberger JM. End-stage primary biliary cirrhosis in a first generation migrant south Asian population. Eur. J. Gastroenterol. Hepatol. 8: 663-666 (1996).

• Anderson CD, Pierce J, Nicoud I, Belous A, Knox CD, Chari RS. Modulation of mitochondrial calcium management attenuates hepatic warm ischemia-reperfusion injury. Liver Transpl. 11: 663-668 (2005). *

• Andre J, Cimaz R, Ranchin B, Galambrun C, Bertrand Y, Bouvier R, Rieux-Laucat F, Trescol-Biemont MC, Cochat P, Bonnefoy-Berard N. Overexpression of the antiapoptotic gene Bfl-1 in B cells from patients with familial systemic lupus erythematosus. Lupus 16: 95-100 (2007). *

• Anekonda TS, Adamus G. Resveratrol prevents antibody-induced apoptotic death of retinal cells through upregulation of Sirt1 and Ku70. BMC Res. Notes 1: 122 (2008). *

• Angulo P. Use of ursodeoxycholic acid in patients with liver disease. Curr. Gastroenterol. Rep. 4: 37-44 (2002).

• Antigny F, Girardin N, Raveau D, Frieden M, Becq F, Vandebrouck C. Dysfunction of mitochondria Ca(2+) uptake in cystic fibrosis airway epithelial cells. Mitochondrion 9: 232-241 (2009).

• Antonio Olivera M, Zapata L, Kaplan M, Bobadilla J, Corte G, de la Mora G, Uribe M, Cabiedes J, Dehesa M, Kershenobich D. Prevalence of antinuclear antibodies in primary biliary cirrhosis, with negative antimitochondrial antibodies. Rev. Gastroenterol. Mex. 58: 220-222 (1993).

• Antunes F, Boveris A, Cadenas E. On the mechanism and biology of cytochrome oxidase inhibition by nitric oxide. Proc. Natl. Acad. Sci. U.S.A. 101: 16774-16779 (2004). *

• Aoki A, Kirino Y, Ishigatsubo Y, Senuma A, Nagaoka S. Liver involvement in primary Sjogren's syndrome. Nihon Rinsho Meneki Gakkai Kaishi 27: 397-401 (2004).

• Arbour L, Rupps R, Field L, Ross P, Erikson A, Henderson H, Hill W, Yoshida E. Characteristics of primary biliary cirrhosis in British Columbia's First Nations population. Can. J. Gastroenterol. 19: 305-310 (2005). *

• Arcavi M, Orfus G. Prevalence of antinuclear envelope antibodies and their isotypes in sera positive for antinuclear antibodies. Medicina (B. Aires) 66: 327-331 (2006).

• Archimandritis A, Tjivras M, Tsirantonaki M, Hatzis G, Delladetsima I. Sjogren's syndrome with antimitochondrial antibody-negative primary biliary cirrhosis: a case of autoimmune cholangitis. J. Clin. Gastroenterol. 20: 268-270 (1995).

• Arcos-Burgos M, Palacio LG, Mora O, Sanchez JL, Jimenez M, Jimenez I. Molecular genetic aspects of the susceptibility to develop idiopathic epilepsy. Rev. Neurol. 30: 173-177 (2000).

• Aronis A, Aharoni-Simon M, Madar Z, Tirosh O. Triacylglycerol-induced impairment in mitochondrial biogenesis and function in J774.2 and mouse peritoneal macrophage foam cells. Arch. Biochem. Biophys. 492: 74-81 (2009). *

• Arundel C, Lewis JH. Drug-induced liver disease in 2006. Curr. Opin. Gastroenterol. 23: 244-254 (2007).

• Arya R, Mallik M, Lakhotia SC. Heat shock genes - integrating cell survival and death. J. Biosci. 32: 595-610 (2007). *

• Assassi S, Fritzler MJ, Arnett FC, Norman GL, Shah KR, Gourh P, Manek N, Perry M, Ganesh D, Rahbar MH, Mayes MD. Primary biliary cirrhosis (PBC), PBC autoantibodies, and hepatic parameter abnormalities in a large population of systemic sclerosis patients. J. Rheumatol. 36: 2250-2256 (2009).

• Atzeni F, Doria A, Ghirardello A, Villalta D, Zampieri S, Carrabba M, Sarzi-Puttini P. Organ-specific autoantibodies in patients with rheumatoid arthritis treated with adalimumab: a prospective long-term follow-up. Autoimmunity 41: 87-91 (2008).

• Autiero I, Costantini S, Colonna G. Human Sirt-1: molecular modeling and structure-function relationships of an unordered protein. PLoS One 4: e7350 (2009). *

• Avigan MI, Adamson G, Hoofnagle JH, Jones EA. The in vitro production of antibodies to mitochondrial antigens by peripheral blood mononuclear cells from patients with primary biliary cirrhosis. Hepatology 6: 999-1004 (1986).

• Azzaroli F, Mehal W, Soroka CJ, Wang L, Lee J, Crispe N, Boyer JL. Ursodeoxycholic acid diminishes Fas-ligand-induced apoptosis in mouse hepatocytes. Hepatology 36: 49-54 (2002). *

• Babb RR. Chronic liver disease. The scope of causes and treatments. Postgrad. Med. 91: 89-96 (1992).

• Bajt ML, Ho YS, Vonderfecht SL, Jaeschke H. Reactive oxygen as modulator of TNF and Fas receptor-mediated apoptosis in vivo: studies with glutathione peroxidase-deficient mice. Antioxid. Redox Signal. 4: 733-740 (2002).

• Balan V, Batts KP, Porayko MK, Krom RA, Ludwig J, Wiesner RH. Histological evidence for recurrence of primary biliary cirrhosis after liver transplantation. Hepatology 18: 1392-1398 (1993).

• Balistreri WF. Genetic liver disease. 54th Annual Meeting of the American Association for the Study of Liver Diseases (2003).

• Bandin O, Courvalin JC, Poupon R, Dubel L, Homberg JC, Johanet C. Specificity and sensitivity of gp210 autoantibodies detected using an enzyme-linked immunosorbent assay and a synthetic polypeptide in the diagnosis of primary biliary cirrhosis. Hepatology 23: 1020-1024 (1996). *

• Bar-Dayan Y, Gershwin ME, Levi Y, Amital H, Shoenfeld Y. Primary biliary cirrhosis. Is (and how much of) the pathology preventible? Immunol. Res. 18: 117-123 (1998).

• Barak V, Selmi C, Schlesinger M, Blank M, Agmon-Levin N, Kalickman I, Gershwin ME, Shoenfeld Y. Serum inflammatory cytokines, complement components, and soluble interleukin 2 receptor in primary biliary cirrhosis. J. Autoimmun. 33: 178-182 (2009).

• Bargou I, Mankai A, Jamaa A, Ben Jazia I, Skandrani K, Sfar H, Baccouche A, Ajmi S, Letaief A, Fabien N, Jeddi M, Ghedira I. Detection of M2 antimitochondrial antibodies by dot blot assay is more specific than by enzyme linked immunosorbent assay. Pathol. Biol. (Paris) 56: 10-14 (2008).

• Barnes BJ, Richards J, Mancl M, Hanash S, Beretta L, Pitha PM. Global and distinct targets of IRF-5 and IRF-7 during innate response to viral infection. J. Biol. Chem. 279: 45194-45207 (2004). *

• Barnes DS. Intrahepatic cholestatic liver disease. http://www.clevelandclinicmeded.com/diseasemanagement/gastro/intrahepatic/intrahepatic1.htm (2002). *

• Bassendine MF. Infectious etiology of primary biliary cirrhosis (PBC). Falk Symposium 142: GASTROENTEROLOGY WEEK FREIBURG 2004 (Part I) AUTOIMMUNE LIVER DISEASE, Freiburg (Germany), October 12 - 13, 2004, pp. 45-46 (2004). *

• Bassendine MF, Jones DE, Yeaman SJ. Biochemistry and autoimmune response to the 2-oxoacid dehydrogenase complexes in primary biliary cirrhosis. Semin. Liver Dis. 17: 49-60 (1997).

• Batts KP, Wang X. Recurrence of primary biliary cirrhosis, autoimmune cholangitis and primary sclerosing cholangitis after liver transplantation. Clin. Liver Dis. 2: 421-435, xi (1998).

• Bauer A, Habior A. Measurement of gp210 autoantibodies in sera of patients with primary biliary cirrhosis. J. Clin. Lab. Anal. 21: 227-231 (2007).

• Bauerova K, Kucharska J, Mihalova D, Navarova J, Gvozdjakova A, Sumbalova Z. Effect of coenzyme Q(10) supplementation in the rat model of adjuvant arthritis. Biomed. Pap. Med. Fac. Univ. Palacky Olomouc. Czech. Repub. 149: 501-503 (2005).

• Baum H. Mitochondrial antigens, molecular mimicry and autoimmune disease. Biochim. Biophys. Acta 1271: 111-121 (1995).

• Bayat A, Walter J, Lambe H, Watson JS, Stanley JK, Marino M, Ferguson MW, Ollier WE. Identification of a novel mitochondrial mutation in Dupuytren's disease using multiplex DHPLC. Plast. Reconstr. Surg. 115: 134-141 (2005).

• Bean P, Sutphin MS, Liu Y, Anton R, Reynolds TB, Shoenfeld Y, Peter JB. Carbohydrate-deficient transferrin and false-positive results for alcohol abuse in primary biliary cirrhosis: differential diagnosis by detection of mitochondrial autoantibodies. Clin. Chem. 41: 858-861 (1995).

• Beckebaum S, Cicinnati V, Broelsch CE. Liver transplantation, rejection and disease recurrence. Falk Symposium 136. XII FALK LIVER WEEK 2003 (Part I) In Honour of Hans Popper’s 100th Birthday. Cholestatic Liver Diseases: Therapeutical Options and Perspectives. Freiburg (Germany). p. 55-56. October 15-16 (2003). *

• Beleznay Z, Regenass S. Diagnostics of autoimmune diseases. Ther. Umsch. 65: 529-537 (2008).

• Bellecave P, Sarasin-Filipowicz M, Donze O, Kennel A, Gouttenoire J, Meylan E, Terracciano L, Tschopp J, Sarrazin C, Berg T, Moradpour D, Heim MH. Cleavage of mitochondrial antiviral signaling protein in the liver of patients with chronic hepatitis C correlates with a reduced activation of the endogenous interferon system. Hepatology Nov 13 [Epub ahead of print] (2009). *

• Beltran B, Nos P, Dasi F, Iborra M, Bastida G, Martinez M, O'Connor JE, Saez G, Moret I, Ponce J. Mitochondrial dysfunction, persistent oxidative damage, and catalase inhibition in immune cells of naive and treated Crohn's disease. Inflamm. Bowel Dis. 16: 76-86 (2010).

• Bensa C, Boscagli A, Bourg V, Bensaken S, Chanalet S, Lebrun C. Recurrent aseptic meningitis associated with primary biliary cirrhosis. Rev. Neurol. (Paris) 161: 87-91 (2005).

• Benson GD, Kikuchi K, Miyakawa H, Tanaka A, Watnik MR, Gershwin ME. Serial analysis of antimitochondrial antibody in patients with primary biliary cirrhosis. Clin. Dev. Immunol. 11: 129-133 (2004). *

• Berg CP, Kannan TR, Klein R, Gregor M, Baseman JB, Wesselborg S, Lauber K, Stein GM. Mycoplasma antigens as a possible trigger for the induction of antimitochondrial antibodies in primary biliary cirrhosis. Liver Int. 29: 797-809 (2009). *

• Berg CP, Stein GM, Klein R, Pascu M, Berg T, Kammer W, Priemer M, Nordheim A, Schulze-Osthoff K, Gregor M, Wesselborg S, Berg PA. Demonstration of PDC-E1 subunits as major antigens in the complement-fixing fraction M4 and re-evaluation of PDC-E1-specific antibodies in PBC patients. Liver Int. 26: 846-855 (2006). *

• Berg PA. Autoimmunity in primary biliary cirrhosis: an alternative view at initiation and function of anti-mitochondrial autoantibodies. J. Hepatol. 50: 827-828 (2009). *

• Berg PA, Binder T, Lindner H, Bannaski H, Maas D, Henning H, Brugel H. Heterogenicity of mitochondrial antibodies. Dtsch. Med. Wochenschr. 100: 1123-1127 (1975).

• Berg PA, Doniach D, Roitt IM. Mitochondrial antibodies in primary biliary cirrhosis. I. Localization of the antigen to mitochondrial membranes. J. Exp. Med. 126: 277-290 (1967). *

• Berg PA, Klein R. Antimitochondrial antibodies in primary biliary cirrhosis and other disorders: definition and clinical relevance. Dig. Dis. 10: 85-101 (1992).

• Berg PA, Klein R. Mitochondrial antigen/antibody systems in primary biliary cirrhosis: revisited. Liver 15: 281-292 (1995).

• Berg PA, Klein R. Antimitochondrial antibodies in primary biliary cirrhosis. A clue to its etiopathogenesis? J. Hepatol. 15: 6-9 (1992).

• Berg PA, Klein R. Mitochondrial antigens and autoantibodies: from anti-M1 to anti-M9. Klin. Wochenschr. 64: 897-909 (1986).

• Berg PA, Klein R. Autoimmune hepatitis and overlap syndrome: diagnosis. Schweiz. Rundsch. Med. Prax. 91: 1339-1346 (2002).

• Berg PA, Klein R. Heterogeneity of antimitochondrial antibodies. Semin. Liver Dis. 9: 103-116 (1989).

• Berg PA, Klein R. Immunology of primary biliary cirrhosis. Baillieres Clin. Gastroenterol. 1: 675-706 (1987).

• Berg PA, Klein R, Lindenborn-Fotinos J. Antimitochondrial antibodies in primary biliary cirrhosis. J. Hepatol. 2: 123-131 (1986).

• Berg PA, Klein R, Lindenborn-Fotinos J, Kloppel W. ATPase-associated antigen (M2): marker antigen for serological diagnosis of primary biliary cirrhosis. Lancet 2: 1423-1426 (1982).

• Berg PA, Muscatello U, Doniach D, Roitt IM. Demonstration of antibodies against the inner mitochondrial membrane in the serum of patients with primary biliary liver cirrhosis. Verh. Dtsch. Ges. Inn. Med. 74: 527-529 (1968).

• Berg PA, Muscatello U, Horne RW, Roitt IM, Doniach D. Mitochondrial antibodies in primary biliary cirrhosis. II. The complement fixing antigen as a component of mitochondrial inner membranes. Br. J. Exp. Pathol. 50: 200-208 (1969).

• Berg PA, Wiedmann KH, Sayers T, Kloppel G, Lindner H. Serological classification of chronic cholestatic liver disease by the use of two different types of antimitochondrial antibodies. Lancet 2: 1329-1332 (1980).

• Bergasa NV. Primary biliary cirrhosis in patients with breast cancer: studying the link. Med. Hypotheses 50: 465-472 (1998).

• Bergasa NV, Mason A, Floreani A, Heathcote J, Swain MG, Jones DE, Lindor KM, Bassendine MF, Worman HJ. Primary biliary cirrhosis: report of a focus study group. Hepatology 40: 1013-1020 (2004). *

• Berthiaume F, MacDonald AD, Kang YH, Yarmush ML. Control analysis of mitochondrial metabolism in intact hepatocytes: effect of interleukin-1beta and interleukin-6. Metab. Eng. 5: 108-123 (2003). *

• Besch R, Poeck H, Hohenauer T, Senft D, Hacker G, Berking C, Hornung V, Endres S, Ruzicka T, Rothenfusser S, Hartmann G. Proapoptotic signaling induced by RIG-I and MDA-5 results in type I interferon-independent apoptosis in human melanoma cells. J. Clin. Invest. 119: 2399-2411 (2009). *

• Beuers U. Mechanisms of action of ursodeoxycholic acid for treatment of primary sclerosing cholangitis. Falk Workshop "Primary Sclerosing Cholangitis: Complications and Consequences", Moscow (Russia), June 11, 2006, p. 12 (2006). *

• Beuers U, Rust C. Chapter 22. Overlap syndromes of autoimmune hepatitis with primary biliary cirrhosis and primary sclerosing cholangitis. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 285-291 (2007). *

• Beuers U, Rust C. Overlap syndromes. Semin. Liver Dis. 25: 311-320 (2005).

• Bhat M, Guindi M, Heathcote EJ, Hirschfield GM. Transient development of anti-mitochondrial antibodies accompanies autoimmune hepatitis-sclerosing cholangitis overlap. Gut 58: 152-153 (2009).

• Bianchi FB, Muratori L. Primary and secondary autoimmunity in hepatology. Ann. Ital. Med. Int. 15: 56-62 (2000).

• Biasi D, Caramasch P, Carletto A, Casaril M, Colombari R, Zeminian S, Bambara LM. Sjogren's syndrome associated with autoimmune hepatitis. A case report. Clin. Rheumatol. 16: 409-412 (1997).

• Bittencourt PL, Farias AQ, Abrantes-Lemos CP, Goncalves LL, Goncalves PL, Magalhaes EP, Carrilho FJ, Laudanna AA, Cancado EL. Prevalence of immune disturbances and chronic liver disease in family members of patients with primary biliary cirrhosis. J. Gastroenterol. Hepatol. 19: 873-878 (2004). *

• Bizzaro N, Tampoia M, Villalta D, Platzgummer S, Liguori M, Tozzoli R, Tonutti E. Low specificity of anti-tissue transglutaminase antibodies in patients with primary biliary cirrhosis. J. Clin. Lab. Anal. 20: 184-189 (2006).

• Bjarnason I. Intestinal permeability. Gut 35: S18-S22 (1994). *

• Bjorkland A, Loof L, Mendel-Hartvig I, Totterman TH. Primary biliary cirrhosis. High proportions of B cells in blood and liver tissue produce anti-mitochondrial antibodies of several Ig classes. J. Immunol. 153: 2750-2757 (1994).

• Bjorkland A, Mendel-Hartvig I, Nelson BD, Totterman TH. Primary biliary cirrhosis (PBC): characterization of a monoclonal antibody (PBC-MoAb) having specificity identical with disease-associated autoantibodies. Scand. J. Immunol. 33: 749-753 (1991).

• Bjorkland A, Totterman TH. Is primary biliary cirrhosis an autoimmune disease? Scand. J. Gastroenterol. Suppl. 204: 32-39 (1994).

• Black D, Bird MA, Samson CM, Lyman S, Lange PA, Schrum LW, Qian T, Lemasters JJ, Brenner DA, Rippe RA, Behrns KE. Primary cirrhotic hepatocytes resist TGFbeta-induced apoptosis through a ROS-dependent mechanism. J. Hepatol. 40: 942-951 (2004). *

• Blanco-Barca O, Gomez-Lado C, Rodrigo-Saez E, Curros-Novos C, Briones-Godino P, Eiris-Punal J, Castro-Gago M. Pyruvate dehydrogenase deficit associated to the C515T mutation in exon 6 of the E1alpha gene. Rev. Neurol. 43: 341-345 (2006).

• Blatt NB, Boitano AE, Lyssiotis CA, Opipari AW Jr, Glick GD. Bz-423 superoxide signals apoptosis via selective activation of JNK, Bak, and Bax. Free Radic. Biol. Med. 45: 1232-1242 (2008).

• Bogdanos D, Vergani D, Mieli-Vergani G. Overlap syndromes from pediatrics to adulthood. J. Gastroenterol. Hepatol. 19: S284-S286 (2004). *

• Bogdanos D, Pusl T, Rust C, Vergani D, Beuers U. Primary biliary cirrhosis following Lactobacillus vaccination for recurrent vaginitis. J. Hepatol. 49: 466-473 (2008). *

• Bogdanos DP, Baum H, Butler P, Rigopoulou EI, Davies ET, Ma Y, Burroughs AK, Vergani D. Association between the primary biliary cirrhosis specific anti-sp100 antibodies and recurrent urinary tract infection. Dig. Liver Dis. 35: 801-805 (2003).

• Bogdanos DP, Baum H, Grasso A, Okamoto M, Butler P, Ma Y, Rigopoulou E, Montalto P, Davies ET, Burroughs AK, Vergani D. Microbial mimics are major targets of crossreactivity with human pyruvate dehydrogenase in primary biliary cirrhosis. J. Hepatol. 40: 31-39 (2004). *

• Bogdanos DP, Baum H, Gunsar F, Arioli D, Polymeros D, Ma Y, Burroughs AK, Vergani D. Extensive homology between the major immunodominant mitochondrial antigen in primary biliary cirrhosis and Helicobacter pylori does not lead to immunological cross-reactivity. Scand. J. Gastroenterol. 39: 981-987 (2004). *

• Bogdanos DP, Baum H, Okamoto M, Montalto P, Sharma UC, Rigopoulou EI, Vlachogiannakos J, Ma Y, Burroughs AK, Vergani D. Primary biliary cirrhosis is characterized by IgG3 antibodies cross-reactive with the major mitochondrial autoepitope and its Lactobacillus mimic. Hepatology 42: 458-465 (2005). *

• Bogdanos DP, Baum H, Vergani D. Antimitochondrial and other autoantibodies. Clin. Liver Dis. 7: 759-777, vi (2003).

• Bogdanos DP, Invernizzi P, Mackay IR, Vergani D. Autoimmune liver serology: current diagnostic and clinical challenges. World J. Gastroenterol. 14: 3374-3387 (2008). *

• Bogdanos DP, Liaskos C, Rigopoulou EI, Dalekos GN. Anti-mitochondrial antibodies in patients with systemic lupus erythematosus: revealing the unforeseen. Clin. Chim. Acta 373: 183-184 (2006). *

• Bogdanos DP, Pares A, Baum H, Caballeria L, Rigopoulou EI, Ma Y, Burroughs AK, Rodes J, Vergani D. Disease-specific cross-reactivity between mimicking peptides of heat shock protein of Mycobacterium gordonae and dominant epitope of E2 subunit of pyruvate dehydrogenase is common in Spanish but not British patients with primary biliary cirrhosis. J. Autoimmun. 22: 353-362 (2004).

• Boki KA, Dourakis SP. Polymyositis associated with primary biliary cirrhosis. Clin. Rheumatol. 14: 375-378 (1995).

• Bonaci-Nikolic B, Andrejevic S, Radlovic N, Davidovic I, Sofronic L, Spuran M, Micev M, Nikolic MM. Serological and clinical comparison of children and adults with anti-endomysial antibodies. J. Clin. Immunol. 27: 163-171 (2007). *

• Bondeson J, Veress B, Lindroth Y, Lindgren S. Polymyositis associated with asymptomatic primary biliary cirrhosis. Clin. Exp. Rheumatol. 16: 172-174 (1998).

• Bonis PA, Kaplan M. Methotrexate improves biochemical tests in patients with primary biliary cirrhosis who respond incompletely to ursodiol. Gastroenterology 117: 395-399 (1999). *

• Bonofiglio D, Cione E, Qi H, Pingitore A, Perri M, Catalano S, Vizza D, Panno ML, Genchi G, Fuqua SA, Ando S. Combined low doses of PPARgamma and RXR ligands trigger an intrinsic apoptotic pathway in human breast cancer cells. Am. J. Pathol. 175: 1270-1280 (2009). *

• Borgaonkar MR, Morgan DG. Primary biliary cirrhosis and type II autoimmune polyglandular syndrome. Can. J. Gastroenterol. 13: 767-770 (1999). *

• Bories C, Certin M, Lavergne A, Galian A, Godeau P, Rambaud JC. Primary biliary cirrhosis and sarcoidosis. Association or unique disease? Gastroenterol. Clin. Biol. 8: 851-855 (1984).

• Brailski Kh, Damianov B. Primary biliary cirrhosis. Vutr. Boles. 22: 27-37 (1983).

• Braun S, Berg C, Buck S, Gregor M, Klein R. Catalytic domain of PDC-E2 contains epitopes recognized by antimitochondrial antibodies in primary biliary cirrhosis. World J. Gastroenterol. 16: 973-981 (2010). *

• Brenard R, Geubel AP. Antimitochondrial and antinuclear antibodies in primary biliary cirrhosis: an update in relation to their biochemical characterization and clinical significance. Acta Clin. Belg. 46: 305-312 (1991).

• Brentnall TA. Ursodiol: good drug makes good. Gastroenterology 124: 1139-1140 (2003). *

• Briand JP, Andre C, Tuaillon N, Herve L, Neimark J, Muller S. Multiple autoepitope presentation for specific detection of antibodies in primary biliary cirrhosis. Hepatology 16: 1395-1403 (1992).

• Briceno-Perez C, Briceno-Sanabria L, Vigil-De Gracia P. Prediction and prevention of preeclampsia. Hypertens. Pregnancy 28: 138-155 (2009).

• Bridoux-Henno L, Maggiore G, Johanet C, Fabre M, Vajro P, Dommergues JP, Reinert P, Bernard O. Features and outcome of autoimmune hepatitis type 2 presenting with isolated positivity for anti-liver cytosol antibody. Clin. Gastroenterol. Hepatol. 2: 825-830 (2004). *

• Brodanova M, Perlik F. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis. Cas. Lek. Cesk. 136: 215-220 (1997).

• Brostoff JM, Rashid S, McCrea D. Primary biliary cirrhosis with a normal alkaline phosphatase: a case report. Cases J. 1: 33 (2008). *

• Bruggraber SF, Leung PS, Amano K, Quan C, Kurth MJ, Nantz MH, Benson GD, Van de Water J, Luketic V, Roche TE, Ansari AA, Coppel RL, Gershwin ME. Autoreactivity to lipoate and a conjugated form of lipoate in primary biliary cirrhosis. Gastroenterology 125: 1705-1713 (2003). *

• Brunmair B, Staniek K, Dorig J, Szocs Z, Stadlbauer K, Marian V, Gras F, Anderwald C, Nohl H, Waldhausl W, Furnsinn C. Activation of PPAR-delta in isolated rat skeletal muscle switches fuel preference from glucose to fatty acids. Diabetologia 49: 2713-2722 (2006). *

• Brunner G, Klinge O. A chronic destructive non-suppurative cholangitis-like disease picture with antinuclear antibodies (immunocholangitis). Dtsch. Med. Wochenschr. 112: 1454-1458 (1987).

• Brzezinska-Kolarz B, Undas A, Dyczek A, Musial J. Reynolds syndrome: the combination of scleroderma and primary biliary cirrhosis. Case report. Pol. Arch. Med. Wewn. 105: 231-234 (2001).

• Bubici C, Papa S, Pham CG, Zazzeroni F, Franzoso G. NF-kappaB and JNK: an intricate affair. Cell Cycle 3: 1524-1529 (2004). *

• Bunn CC, McMorrow M. Anti-M4 antibodies measured by a sulphite oxidase ELISA in patients with both anti-centromere and anti-M2 antibodies. Clin. Exp. Immunol. 102: 131-136 (1995). *

• Burt AD. Primary biliary cirrhosis and other ductopenic diseases. Clin. Liver Dis. 6: 363-380 (2002).

• Burt AD. Spectrum of liver pathology in immune mediated liver diseases. J. Gastroenterol. Hepatol. 19: S353-S355 (2004). *

• Buse C, Altmann F, Amann B, Hauck SM, Poulsen Nautrup C, Ueffing M, Stangassinger M, Deeg CA. Discovering novel targets for autoantibodies in dilated cardiomyopathy. Electrophoresis 29: 1325-1332 (2008).

• Butler P, Hamilton-Miller J, Baum H, Burroughs AK. Detection of M2 antibodies in patients with recurrent urinary tract infection using an ELISA and purified PBC specific antigens. Evidence for a molecular mimicry mechanism in the pathogenesis of primary biliary cirrhosis? Biochem. Mol. Biol. Int. 35: 473-485 (1995).

• Butler P, Valle F, Hamilton-Miller JM, Brumfitt W, Baum H, Burroughs AK. M2 mitochondrial antibodies and urinary rough mutant bacteria in patients with primary biliary cirrhosis and in patients with recurrent bacteriuria. J. Hepatol. 17: 408-414 (1993).

• Caballeria L, Pares A, Rodes J. Treatment of primary biliary cirrhosis. Contributions to Science 1: 79-85 (1999). *

• Cabibi D, Tarantino G, Barbaria F, Campione M, Craxi A, Di Marco V. Intrahepatic IgG/IgM plasma cells ratio helps in classifying autoimmune liver diseases. Dig. Liver Dis. Jan 6 [Epub ahead of print] (2010).

• Cable EE, Finn PD, Stebbins JW, Hou J, Ito BR, van Poelje PD, Linemeyer DL, Erion MD. Reduction of hepatic steatosis in rats and mice after treatment with a liver-targeted thyroid hormone receptor agonist. Hepatology 49: 407-417 (2009). *

• Caglar E, Ugurlu S, Ozenoglu A, Can G, Kadioglu P, Dobrucali A. Autoantibody frequency in celiac disease. Clinics (Sao Paulo) 64: 1195-1200 (2009). *

• Calabrese GC, Luczak EN, Estela Roux M. Importance of CCL25 in the attraction of T cells and the role of IL-7 on the signaling pathways in intestinal epithelial cells. Immunobiology Feb 25 [Epub ahead of print] (2009).

• Calabrese V, Lodi R, Tonon C, D'Agata V, Sapienza M, Scapagnini G, Mangiameli A, Pennisi G, Stella AM, Butterfield DA. Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia. J. Neurol. Sci. 233: 145-162 (2005). *

• Caldwell SH, de Freitas LA, Park SH, Moreno ML, Redick JA, Davis CA, Sisson BJ, Patrie JT, Cotrim H, Argo CK, Al-Osaimi A. Intramitochondrial crystalline inclusions in nonalcoholic steatohepatitis. Hepatology 49: 1888-1895 (2009).

• Caldwell SH, Hespenheide EE, Redick JA, Iezzoni JC, Battle EH, Sheppard BL. A pilot study of a thiazolidinedione, troglitazone, in nonalcoholic steatohepatitis. Am. J. Gastroenterol. 96: 519-525 (2001). *

• Caldwell SH, Leung PS, Spivey JR, Prindiville T, de Medina M, Saicheur T, Rowley M, Reddy KR, Coppel R, Jeffers LJ, et al. Antimitochondrial antibodies in kindreds of patients with primary biliary cirrhosis: antimitochondrial antibodies are unique to clinical disease and are absent in asymptomatic family members. Hepatology 16: 899-905 (1992).

• Cales P, Calot M, Voigt JJ, Oksman F, Cassigneul J, Vinel JP, Pascal JP. Familial autoimmune pathology comprising 2 cases of primary biliary cirrhosis. Gastroenterol. Clin. Biol. 7: 777-784 (1983).

• Calmus Y, Poupon R. Ursodeoxycholic acid (UDCA) in the treatment of chronic cholestatic diseases. Biochimie 73: 1335-1338 (1991).

• Cappello F, Conway de Macario E, Di Felice V, Zummo G, Macario AJ. Chlamydia trachomatis infection and anti-Hsp60 immunity: the two sides of the coin. PLoS Pathog. 5: e1000552 (2009). *

• Caramaschi P, Biasi D, Colombatti M, Pieropan S, Martinelli N, Carletto A, Volpe A, Pacor LM, Bambara LM. Anti-TNFalpha therapy in rheumatoid arthritis and autoimmunity. Rheumatol. Int. 26: 209-214 (2006). *

• Carroccio A, Giannitrapani L, Soresi M, Not T, Iacono G, Di Rosa C, Panfili E, Notarbartolo A, Montalto G. Guinea pig transglutaminase immunolinked assay does not predict coeliac disease in patients with chronic liver disease. Gut 49: 506-511 (2001). *

• Cars O, Stenram U, Stromberg A. Alpha-1-antitrypsin deficiency, mitochondrial antibodies and possible primary biliary cirrhosis. A case report and family study. Ups. J. Med. Sci. 80: 93-98 (1975).

• Cassani F, Bianchi FB, Lenzi M, Volta U, Pisi E. Immunomorphological characterisation of antinuclear antibodies in chronic liver disease. J. Clin. Pathol. 38: 801-805 (1985). *

• Castera L, Hatzfeld-Charbonnier AS, Ballot C, Charbonnel F, Dhuiege E, Velu T, Formstecher P, Mortier L, Marchetti P. Apoptosis-related mitochondrial dysfunction defines human monocyte-derived dendritic cells with impaired immuno-stimulatory capacities. J. Cell. Mol. Med. 13: 1321-1335 (2009). *

• Castro RE, Sola S, Ramalho RM, Steer CJ, Rodrigues CM. The bile acid tauroursodeoxycholic acid modulates phosphorylation and translocation of Bad via phosphatidylinositol 3-kinase in glutamate-induced apoptosis of rat cortical neurons. J. Pharmacol. Exp. Ther. 311: 845-852 (2004). *

• Cazanave S, Vadrot N, Tinel M, Berson A, Letteron P, Larosche I, Descatoire V, Feldmann G, Robin MA, Pessayre D. Ibuprofen administration attenuates serum TNF-alpha levels, hepatic glutathione depletion, hepatic apoptosis and mouse mortality after Fas stimulation. Toxicol. Appl. Pharmacol. 231: 336-343 (2008).

• Cecere A, Caiazzo R, Romano C, Gattoni A. Primary biliary cirrhosis. Clin. Ter. 149: 143-150 (1998).

• Cervio E, Volta U, Verri M, Boschi F, Pastoris O, Granito A, Barbara G, Parisi C, Felicani C, Tonini M, De Giorgio R. Sera of patients with celiac disease and neurologic disorders evoke a mitochondrial-dependent apoptosis in vitro. Gastroenterology 133: 195-206 (2007). *

• Cetindere T, Nambiar S, Santourlidis S, Essmann F, Hassan M. Induction of indoleamine 2, 3-dioxygenase by death receptor activation contributes to apoptosis of melanoma cells via mitochondrial damage-dependent ROS accumulation. Cell. Signal. 22: 197-211 (2010). *

• Cha S, Leung PS, Coppel RL, Van de Water J, Ansari AA, Gershwin ME. Heterogeneity of combinatorial human autoantibodies against PDC-E2 and biliary epithelial cells in patients with primary biliary cirrhosis. Hepatology 20: 574-583 (1994).

• Cha S, Leung PS, Gershwin ME, Fletcher MP, Ansari AA, Coppel RL. Combinatorial autoantibodies to dihydrolipoamide acetyltransferase, the major autoantigen of primary biliary cirrhosis. Proc. Natl. Acad. Sci. U.S.A. 90: 2527-2531 (1993). *

• Cha S, Leung PS, Van de Water J, Tsuneyama K, Joplin RE, Ansari AA, Nakanuma Y, Schatz PJ, Cwirla S, Fabris LE, Neuberger JM, Gershwin ME, Coppel RL. Random phage mimotopes recognized by monoclonal antibodies against the pyruvate dehydrogenase complex-E2 (PDC-E2). Proc. Natl. Acad. Sci. U.S.A. 93: 10949-10954 (1996). *

• Chan CY, Huang YS, Wu JC, Lu RH, Hwang SJ, Wang YJ, Lee SD. Detection of antibody to M2 mitochondrial antigen in Chinese patients with primary biliary cirrhosis. Zhonghua Yi Xue Za Zhi (Taipei) 55: 214-218 (1995).

• Chaouat D, Belange G, Gompel H. Polymyalgia rheumatica in primary biliary cirrhosis: value of the search for antimitochondrial antibodies in rhizomelic pseudo-polyarthritis and Horton disease. Rev. Med. Interne. 15: 775-776 (1994).

• Chapman RW. The management of primary sclerosing cholangitis. Curr. Gastroenterol. Rep. 5: 9-17 (2003).

• Charatcharoenwitthaya P, Lindor KD. Current concepts in the pathogenesis of primary biliary cirrhosis. Ann. Hepatol. 4: 161-175 (2005). *

• Chaturvedi RK, Flint Beal M. PPAR: a therapeutic target in Parkinson's disease. J. Neurochem. 106: 506-518 (2008). *

• Chaudiere J, Ferrari-Iliou R. Intracellular antioxidants: from chemical to biochemical mechanisms. Food Chem. Toxicol. 37: 949-962 (1999).

• Chazouilleres O, Andreani T, Legendre C, Poupon R, Darnis F. Nodular regenerative hyperplasia of the liver and primary biliary cirrhosis. A fortuitous association? Gastroenterol. Clin. Biol. 10: 764-766 (1986).

• Chazouilleres O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 28: 296-301 (1998). *

• Chen CC, Meng HC, Chang FY, Yang WG, Ng WW, Lee SD. Characteristics of endoscopic retrograde cholangiopancreatogram in patients with primary biliary cirrhosis. Zhonghua Yi Xue Za Zhi (Taipei) 57: 254-259 (1996).

• Chen J, Lu Y, Lee CH, Li R, Leiter EH, Mathews CE. Commonalities of genetic resistance to spontaneous autoimmune and free radical-mediated diabetes. Free Radic. Biol. Med. 45: 1263-1270 (2008).

• Chen L, Hu L, Chan TH, Tsao GS, Xie D, Huo KK, Fu L, Ma S, Zheng BJ, Guan XY. Chromodomain helicase/adenosine triphosphatase DNA binding protein 1-like (CHD1l) gene suppresses the nucleus-to-mitochondria translocation of nur77 to sustain hepatocellular carcinoma cell survival. Hepatology 50: 122-129 (2009).

• Chen QY, Mackay IR, Fida S, Myers MA, Rowley MJ. Natural and disease associated autoantibodies to the autoantigen, dihydrolipoamide acetyltransferase, recognise different epitopes. J. Autoimmun. 11: 151-161 (1998).

• Chen QY, Rowley MJ, Mackay IR. Antibody to two forms of dihydrolipoamide acetyltransferase (PDC-E2) in primary biliary cirrhosis. Liver 13: 130-135 (1993).

• Chen Y, Yang Y, Miller ML, Shen D, Shertzer HG, Stringer KF, Wang B, Schneider SN, Nebert DW, Dalton TP. Hepatocyte-specific Gclc deletion leads to rapid onset of steatosis with mitochondrial injury and liver failure. Hepatology 45: 1118-1128 (2007). *

• Chernev K, Bakalov V. Clinical-dispensary finding of patients with primary biliary cirrhosis. Vutr. Boles. 24: 42-46 (1985).

• Chester KA, Robson L, Begent RH, Pringle H, Primrose L, Talbot IC, Macpherson AJ, Owen SL, Boxer G, Malcolm AD. In situ and slot hybridization analysis of RNA in colorectal tumours and normal colon shows distinct distributions of mitochondrial sequences. J. Pathol. 162: 309-315 (1990).

• Chevailler A, Saint-Andre JP, Preel JL, Boyer J, Joubaud F, Hurez D. Clinico-immunological profile of hepatic and extrahepatic diseases accompanied by anti-mitochondrial antibodies. Ann. Med. Interne. (Paris) 139: 19-23 (1988).

• Chiang PC, Chien CL, Pan SL, Chen WP, Teng CM, Shen YC, Guh JH. Induction of endoplasmic reticulum stress and apoptosis by a marine prostanoid in human hepatocellular carcinoma. J. Hepatol. 43: 679-686 (2005). *

• Chien RN, Sheen IS, Chen TJ, Liaw YF. A clinicopathological study in primary biliary cirrhosis. J. Formos. Med. Assoc. 91 Suppl. 2: S117-S121 (1992).

• Chiu YH, Hsieh YJ, Liao KW, Peng KC. Preferential promotion of apoptosis of monocytes by Lactobacillus casei rhamnosus soluble factors. Clin. Nutr. Aug 6 [Epub ahead of print] (2009).

• Chiu YH, Macmillan JB, Chen ZJ. RNA polymerase III detects cytosolic DNA and induces type I interferons through the RIG-I pathway. Cell 138: 576-591 (2009). *

• Cho DH, Choi MS, Kim DH, Kim do Y, Shim SG, Lee JH, Koh KC, Paik SW, Yoo BC, Rhee JC. A prospective study on the prevalence and clinical significance of autoantibodies in patients with suspected nonalcholic fatty liver disease. Korean J. Hepatol. 11: 261-267 (2005).

• Cho YJ, Han DS, Kim TY, Jang SJ, Jeon YC, Sohn JH, Lee IH, Park KN. Negative conversion of antimitochondrial antibody in primary biliary cirrhosis: a case of autoimmune cholangitis. J. Korean Med. Sci. 14: 102-106 (1999). *

• Chon CY, Park JY. Primary biliary cirrhosis. Korean J. Hepatol. 12: 364-372 (2006).

• Chou MJ, Lai MY, Lee SL, Tsay GJ. Reactivity of anti-mitochondrial antibodies in primary biliary cirrhosis and systemic sclerosis. J. Formos. Med. Assoc. 91: 1075-1080 (1992).

• Choudhary R, Baker KM, Pan J. All-trans retinoic acid prevents angiotensin II- and mechanical stretch-induced reactive oxygen species generation and cardiomyocyte apoptosis. J. Cell. Physiol. 215: 172-181 (2008). *

• Chowanadisai W, Bauerly KA, Tchaparian E, Wong A, Cortopassi GA, Rucker RB. Pyrroloquinoline quinone stimulates mitochondrial biogenesis through CREB phosphorylation and increased PGC-1alpha expression. J. Biol. Chem. 285: 142-152 (2010). *

• Chuang YH, Lian ZX, Yang GX, Shu SA, Moritoki Y, Ridgway WM, Ansari AA, Kronenberg M, Flavell RA, Gao B, Gershwin ME. Natural killer T cells exacerbate liver injury in a transforming growth factor beta receptor II dominant-negative mouse model of primary biliary cirrhosis. Hepatology 47: 571-580 (2008). *

• Ciechomska IA, Goemans GC, Skepper JN, Tolkovsky AM. Bcl-2 complexed with Beclin-1 maintains full anti-apoptotic function. Oncogene 28: 2128-2141 (2009). *

• Clapham JC, Arch JR. Thermogenic and metabolic antiobesity drugs: rationale and opportunities. Diabetes Obes. Metab. 9: 259-275 (2007). *

• Clemente MG, Obermayer-Straub P, Meloni A, Strassburg CP, Arangino V, Tukey RH, De Virgiliis S, Manns MP. Cytochrome P450 1A2 is a hepatic autoantigen in autoimmune polyglandular syndrome type 1. J. Clin. Endocrinol. Metab. 82: 1353-1361 (1997). *

• Colombato LA, Alvarez F, Cote J, Huet PM. Autoimmune cholangiopathy: the result of consecutive primary biliary cirrhosis and autoimmune hepatitis? Gastroenterology 107: 1839-1843 (1994).

• Comay D, Cauch-Dudek K, Hemphill D, Diamandis E, Wanless I, Heathcote EJ. Are antibodies to carbonic anhydrase II specific for anti-mitochondrial antibody-negative primary biliary cirrhosis? Dig. Dis. Sci. 45: 2018-2021 (2000). *

• Conrad DJ, Stenbit AE, Zettner EM, Wick I, Eckhardt C, Hardiman G. Frequency of mitochondrial 12S ribosomal RNA variants in an adult cystic fibrosis population. Pharmacogenet. Genomics 18: 1095-1102 (2008).

• Constantin-Teodosiu D, Baker DJ, Constantin D, Greenhaff PL. PPARdelta agonism inhibits skeletal muscle PDC activity, mitochondrial ATP production and force generation during prolonged contraction. J. Physiol. 587: 231-239 (2009). *

• Constantinou C, Papas A, Constantinou AI. Vitamin E and cancer: an insight into the anticancer activities of vitamin E isomers and analogs. Int. J. Cancer 123: 739-752 (2008). *

• Coppel RL, Gershwin ME. Primary biliary cirrhosis: the molecule and the mimic. Immunol. Rev. 144: 17-49 (1995).

• Coppel RL, McNeilage LJ, Surh CD, Van de Water J, Spithill TW, Whittingham S, Gershwin ME. Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase. Proc. Natl. Acad. Sci. U.S.A. 85: 7317-7321 (1988). *

• Costa CD, Hammes TO, Rohden F, Margis R, Bortolotto JW, Padoin AV, Mottin CC, Guaragna RM. SIRT1 transcription is decreased in visceral adipose tissue of morbidly obese patients with severe hepatic steatosis. Obes. Surg. Dec 22 [Epub ahead of print] (2009).

• Courvalin JC, Worman HJ. Nuclear envelope protein autoantibodies in primary biliary cirrhosis. Semin. Liver Dis. 17: 79-90 (1997).

• Covelli M, Favia I, Marrone M, Iannone F, Gentile A, Lastilla G, Lapadula G. Cutaneous lesions as presenting symptoms of primary biliary cirrhosis: an undifferentiated connective tissue disease-like onset. Clin. Rheumatol. 25: 919-922 (2006). *

• Cowell RM, Blake KR, Russell JW. Localization of the transcriptional coactivator PGC-1alpha to GABAergic neurons during maturation of the rat brain. J. Comp. Neurol. 502: 1-18 (2007).

• Crabb DW. Pathogenesis of alcoholic liver disease: newer mechanisms of injury. Keio. J. Med. 48: 184-188 (1999).

• Crippa G, Cagnoni C, Castelli A, Concesi C, Girometta S, Pancotti D, Sverzellati E, Tacchini G, Pierfranceschi MG, Carrara GC. Prolonged treatment with ursodeoxycholic acid for primary biliary cirrhosis. Clin. Ter. 146: 367-372 (1995).

• Crispin-Acuna JC, Remes-Troche JM, Uribe M. Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjogren's syndrome. Ann. Hepatol. 6: 150-155 (2007). *

• Crivellari D, Cannizzaro R, Galligioni E, Valentini M, Monfardini S, Castiglione M. Adjuvant therapy for primary breast cancer in a female patient affected by autoimmune liver disease: does it cure breast cancer and primary biliary cirrhosis? Tumori. 78: 395-396 (1992).

• Csepregi A, Obermayer-Straub P, Kneip S, Kayser A, Loges S, Schmidt E, Nemesanszky E, Szalay F, Manns MP, Strassburg CP. Characterization of a lipoyl domain-independent B-cell autoepitope on the human branched-chain acyltransferase in primary biliary cirrhosis and overlap syndrome with autoimmune hepatitis. Clin. Dev. Immunol. 10: 173-181 (2003). *

• Csepregi A, Szodoray P, Zeher M. Do autoantibodies predict autoimmune liver disease in primary Sjogren's syndrome? Data of 180 patients upon a 5 year follow-up. Scand. J. Immunol. 56: 623-629 (2002). *

• Cukovic-Cavka S. Primary biliary cirrhosis. Acta Med. Croatica 57: 221-225 (2003).

• Cuzzocrea S. Role of nitric oxide and reactive oxygen species in arthritis. Curr. Pharm. Des. 12: 3551-3570 (2006).

• Czaja AJ. Autoimmune hepatitis - approach to diagnosis. MedGenMed. 8: 55 (2006). *

• Czaja AJ. Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a foray across diagnostic boundaries. J. Hepatol. 44: 251-252 (2006). *

• Czaja AJ. Autoantibodies in autoimmune liver disease. Adv. Clin. Chem. 40: 127-164 (2005).

• Czaja AJ. Autoimmune liver disease. Curr. Opin. Gastroenterol. 23: 255-262 (2007).

• Czaja AJ. Variant forms of autoimmune hepatitis. Curr. Gastroenterol. Rep. 1: 63-70 (1999).

• Czaja AJ, Bayraktar Y. Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment. World J. Gastroenterol. 15: 2314-2328 (2009). *

• Czaja AJ, Carpenter HA, Manns MP. Antibodies to soluble liver antigen, P450IID6, and mitochondrial complexes in chronic hepatitis. Gastroenterology 105: 1522-1528 (1993).

• Czaja AJ, Carpenter HA, Santrach PJ, Moore SB. Genetic predispositions for immunological features in chronic liver diseases other than autoimmune hepatitis. J. Hepatol. 24: 52-59 (1996). *

• Czaja AJ, Manns MP, Homburger HA. Frequency and significance of antibodies to liver/kidney microsome type 1 in adults with chronic active hepatitis. Gastroenterology 103: 1290-1295 (1992).

• Czaja AJ, Norman GL. Autoantibodies in the diagnosis and management of liver disease. J. Clin. Gastroenterol. 37: 315-329 (2003).

• Czompoly T, Olasz K, Nyarady Z, Simon D, Bovari J, Nemeth P. Detailed analyses of antibodies recognizing mitochondrial antigens suggest similar or identical mechanism for production of natural antibodies and natural autoantibodies. Autoimmun. Rev. 7: 463-467 (2008).

• Czompoly T, Olasz K, Simon D, Nyarady Z, Palinkas L, Czirjak L, Berki T, Nemeth P. A possible new bridge between innate and adaptive immunity: are the anti-mitochondrial citrate synthase autoantibodies components of the natural antibody network? Mol. Immunol. 43: 1761-1768 (2006). *

• D'Amico E, Paroli M, Fratelli V, Palazzi C, Barnaba V, Callea F, Consoli G. Primary biliary cirrhosis induced by interferon-alpha therapy for hepatitis C virus infection. Dig. Dis. Sci. 40: 2113-2116 (1995).

• da Rosa Utiyama SR, da Silva Kotze LM, Nisihara RM, Carvalho RF, de Carvalho EG, de Sena MG, de Messias Reason IJ. Spectrum of autoantibodies in celiac patients and relatives. Dig. Dis. Sci. 46: 2624-2630 (2001). *

• da Rosa Utiyama SR, Nisihara RM, Nass FR, Oliveira NP, Fiedler PT, de Messias-Reason IT. Autoantibodies in patients with Down syndrome: early senescence of the immune system or precocious markers for immunological diseases? J. Paediatr. Child. Health 44: 182-186 (2008). *

• Dahlan Y, Smith L, Simmonds D, Jewell LD, Wanless I, Heathcote EJ, Bain VG. Pediatric-onset primary biliary cirrhosis. Gastroenterology 125: 1476-1479 (2003). *

• Dahnrich C, Pares A, Caballeria L, Rosemann A, Schlumberger W, Probst C, Mytilinaiou M, Bogdanos D, Vergani D, Stocker W, Komorowski L. New ELISA for detecting primary biliary cirrhosis-specific antimitochondrial antibodies. Clin. Chem. 55: 978-985 (2009). *

• Dai Y, Liang YH, Xie PY, Chen BW, Liu XG. Clinical analysis of primary biliary cirrhosis: a report of 42 cases. Beijing Da Xue Xue Bao 37: 410-414 (2005).

• Das KM. Immunopathogenesis of primary sclerosing cholangitis: possible role of a shared colonic and biliary epithelial antigen. J. Gastroenterol. Hepatol. 19: S290-S294 (2004). *

• Das UN. A defect in the activity of Delta(6) and Delta(5) desaturases may be a factor in the initiation and progression of atherosclerosis. Prostaglandins Leukot. Essent. Fatty Acids 76: 251-268 (2007). *

• Dash A, Kragel P. Primary biliary cirrhosis in an elderly man with normal liver tests. South. Med. J. 102: 1272-1274 (2009).

• Davalos M, Roman R, Bustios C, Zumaeta E, Yabar A. Autoimmune hepatitis: clinical forms and related factors to their response to treatment. Rev. Gastroenterol. Peru 24: 305-313 (2004).

• Davidson MH, Yannicelli HD. New concepts in dyslipidemia in the metabolic syndrome and diabetes. Metab. Syndr. Relat. Disord. 4: 299-314 (2006).

• Davis LM, Pei Z, Trush MA, Cheskin LJ, Contoreggi C, McCullough K, Watkins PA, Moran TH. Bromocriptine reduces steatosis in obese rodent models. J. Hepatol. 45: 439-444 (2006). *

• Davis LM, Rho JM, Sullivan PG. UCP-mediated free fatty acid uncoupling of isolated cortical mitochondria from fasted animals: correlations to dietary modulations. Epilepsia 49 Suppl. 8: 117-119 (2008).

• Davis PA, Leung P, Manns M, Kaplan M, Munoz SJ, Gorin FA, Dickson ER, Krawitt E, Coppel R, Gershwin ME. M4 and M9 antibodies in the overlap syndrome of primary biliary cirrhosis and chronic active hepatitis: epitopes or epiphenomena? Hepatology 16: 1128-1136 (1992).

• de Giorgio R, Volta U, Stanghellini V, Cogliandro RF, Barbara G, Corinaldesi R, Towns R, Guo C, Hong S, Wiley JW. Neurogenic chronic intestinal pseudo-obstruction: antineuronal antibody-mediated activation of autophagy via Fas. Gastroenterology 135: 601-609 (2008). *

• de Oliveira MR, Oliveira MW, Da Rocha RF, Moreira JC. Vitamin A supplementation at pharmacological doses induces nitrosative stress on the hypothalamus of adult Wistar rats. Chem. Biol. Interact. 180: 407-413 (2009). *

• de Oliveira MR, Oliveira MW, Hoff ML, Behr GA, da Rocha RF, Moreira JC. Evaluation of redox and bioenergetics states in the liver of vitamin A-treated rats. Eur. J. Pharmacol. 610: 99-105 (2009). *

• de Oliveira MR, Oliveira MW, Lorenzi R, Fagundes da Rocha R, Fonseca Moreira JC. Short-term vitamin A supplementation at therapeutic doses induces a pro-oxidative state in the hepatic environment and facilitates calcium-ion-induced oxidative stress in rat liver mitochondria independently from permeability transition pore formation … Cell. Biol. Toxicol. 25: 545-560 (2009). *

• De Rosa S, Cirillo P, Paglia A, Sasso L, Di Palma V, Chiariello M. Reactive oxygen species and antioxidants in the pathophysiology of cardiovascular disease: does the actual knowledge justify a clinical approach? Curr. Vasc. Pharmacol. Jan 1 [Epub ahead of print] (2010).

• del Olmo L, Moreira VF, Segi J, Gonzalez Martin JA, Cano A, Ruiz del Arbol L. Significance of antimitochondrial antibodies in a Spanish hospital population. Rev. Clin. Esp. 181: 492-495 (1987).

• Delgado M, Singh S, De Haro S, Master S, Ponpuak M, Dinkins C, Ornatowski W, Vergne I, Deretic V. Autophagy and pattern recognition receptors in innate immunity. Immunol. Rev. 227: 189-202 (2009). *

• Demirag MD, Goker B, Haznedaroglu S, Ozturk MA, Karakan T, Kuruoglu R. Osteomalacic myopathy associated with coexisting coeliac disease and primary biliary cirrhosis. Med. Princ. Pract. 17: 425-428 (2008).

• Deng A, Yao D, Chen S, Liu C, Kang J, Zhou Y, Yu H, Qian C, Wu C, Jiang T, Zhong R, Pei G. beta-Arrestin 1 contributes to primary biliary cirrhosis. Clin. Sci. (Lond.) Dec 21 [Epub ahead of print] (2009). *

• Deretic V. Autophagy in infection. Curr. Opin. Cell Biol. Jan 28 [Epub ahead of print] (2010).

• Dewitte-Orr SJ, Mehta DR, Collins SE, Suthar M, Gale M Jr, Mossman KL. Long double-stranded RNA induces an antiviral response independent of IFN regulatory factor 3, IFN-beta promoter stimulator 1, and IFN. J. Immunol. 183: 6545-6553 (2009).

• Dhote R, Christoforov B, Weill B, Menkes CJ. Joint and systemic manifestations related to type 2 anti-mitochondrial antibodies. Ann. Med. Interne. (Paris) 146: 536-540 (1995).

• Diehl C, Lipozencic J, Ledic-Drvar D. The basis of topical superoxide dismutase antipruritic activity. Acta Dermatovenerol. Croat. 17: 25-39 (2009).

• Dighiero G, Magnac C, de Saint Martin J, Abuaf N. Detection of anti-mitochondrial antibodies by ELISA and Western-blot techniques and identification by one and two-dimensional gel electrophoresis of M2 target antigens. Clin. Exp. Immunol. 70: 640-648 (1987). *

• Ding WX, Ni HM, DiFrancesca D, Stolz DB, Yin XM. Bid-dependent generation of oxygen radicals promotes death receptor activation-induced apoptosis in murine hepatocytes. Hepatology 40: 403-413 (2004). *

• Dionne S, Levy E, Levesque D, Seidman EG. PPARgamma ligand 15-deoxy-delta 12,14-prostaglandin J2 sensitizes human colon carcinoma cells to TWEAK-induced apoptosis. Anticancer Res. 30: 157-166 (2010).

• Diu A, Moebius U, Ferradini L, Genevee C, Roman-Roman S, Claudon M, Delorme D, Meuer S, Hercend T, Praz F. Limited T-cell receptor diversity in liver-infiltrating lymphocytes from patients with primary biliary cirrhosis. J. Autoimmun. 6: 611-619 (1993).

• Dobbs RJ, Dobbs SM, Weller C, Charlett A, Bjarnason IT, Curry A, Ellis DS, Ibrahim MA, McCrossan MV, O'Donohue J, Owen RJ, Oxlade NL, Price AB, Sanderson JD, Sudhanva M, Williams J. Helicobacter hypothesis for idiopathic parkinsonism: before and beyond. Helicobacter 13: 309-322 (2008). *

• Doering J, Begue B, Lentze MJ, Rieux-Laucat F, Goulet O, Schmitz J, Cerf-Bensussan N, Ruemmele FM. Induction of T lymphocyte apoptosis by sulphasalazine in patients with Crohn’s disease. Gut 53: 1632-1638 (2004). *

• Dohmen K, Mizuta T, Nakamuta M, Shimohashi N, Ishibashi H, Yamamoto K. Fenofibrate for patients with asymptomatic primary biliary cirrhosis. World J. Gastroenterol. 10: 894-898 (2004). *

• Domercq M, Sanchez-Gomez MV, Sherwin C, Etxebarria E, Fern R, Matute C. System xc- and glutamate transporter inhibition mediates microglial toxicity to oligodendrocytes. J. Immunol. 178: 6549-6556 (2007). *

• Douglas JG, Finlayson ND. Are increased individual susceptibility and environmental factors both necessary for the development of primary biliary cirrhosis? Br. Med. J. 2: 419-420 (1979). *

• Drebber U, Mueller JJ, Klein E, Kasper HU, Schulze F, Schardt K, Quasdorff M, Schulte S, Odenthal M, Dienes HP. Liver biopsy in primary biliary cirrhosis: clinicopathological data and stage. Pathol. Int. 59: 546-554 (2009). *

• Dubel L, Farges O, Bismuth H, Sebagh M, Homberg JC, Johanet C. Kinetics of anti-M2 antibodies after liver transplantation for primary biliary cirrhosis. J. Hepatol. 23: 674-680 (1995). *

• Dubel L, Tanaka A, Leung PS, Van de Water J, Coppel R, Roche T, Johanet C, Motokawa Y, Ansari A, Gershwin ME. Autoepitope mapping and reactivity of autoantibodies to the dihydrolipoamide dehydrogenase-binding protein (E3BP) and the glycine cleavage proteins in primary biliary cirrhosis. Hepatology 29: 1013-1018 (1999). *

• Duclos-Vallee JC, Hadengue A, Ganne-Carrie N, Robin E, Degott C, Erlinger S. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Corticoresistance and effective treatment by cyclosporine A. Dig. Dis. Sci. 40: 1069-1073 (1995).

• Duclos-Vallee JC, Sebagh M. Recurrence of autoimmune disease, primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune hepatitis after liver transplantation. Liver Transpl. 15: S25-S34 (2009).

• Duda MK, O'Shea KM, Stanley WC. Omega-3 polyunsaturated fatty acid supplementation for the treatment of heart failure: mechanisms and clinical potential. Cardiovasc. Res. 84: 33-41 (2009).

• Duro D, Duggan C, Valim C, Bechard L, Fitzgibbons S, Jaksic T, Yu YM. Novel intravenous (13)C-methionine breath test as a measure of liver function in children with short bowel syndrome. J. Pediatr. Surg. 44: 236-240 (2009).

• Dyroy E, Rost TH, Pettersen RJ, Halvorsen B, Gudbrandsen OA, Ueland T, Muna Z, Muller F, Nordrehaug JE, Aukrust P, Berge RK. Tetradecylselenoacetic acid, a PPAR ligand with antioxidant, antiinflammatory, and hypolipidemic properties. Arterioscler. Thromb. Vasc. Biol. 27: 628-634 (2007). *

• Eeva J, Nuutinen U, Ropponen A, Matto M, Eray M, Pellinen R, Wahlfors J, Pelkonen J. Feedback regulation of mitochondria by caspase-9 in the B cell receptor-mediated apoptosis. Scand. J. Immunol. 70: 574-583 (2009). *

• Eklund A, Stjernberg N, Uddenfeldt P, Danielsson A. Kveim test and mitochondrial antibodies in the differential diagnosis between primary biliary cirrhosis and sarcoidosis. Sarcoidosis 2: 38-41 (1985).

• El Kebir D, Jozsef L, Pan W, Wang L, Petasis NA, Serhan CN, Filep JG. 15-epi-Lipoxin A4 inhibits myeloperoxidase signaling and enhances resolution of acute lung injury. Am. J. Respir. Crit. Care Med. 180: 311-319 (2009). *

• Elias E. Hepatology highlights. Hepatology 39: 1467-1468 (2004). *

• Elkhalifa MY, Kiechle FL, Gordon SC, Chen J, Poulik MD. A flow cytometric method to detect anti-pyruvate dehydrogenase antibody in primary biliary cirrhosis. Am. J. Clin. Pathol. 97: 202-208 (1992).

• Elsakka NE, Webster NR, Galley HF. Polymorphism in the manganese superoxide dismutase gene. Free Radic. Res. 41: 770-778 (2007). *

• Erol A. PPARalpha activators may be good candidates as antiaging agents. Med. Hypotheses 65: 35-38 (2005).

• Etxagibel A, Julia MR, Brotons A, Company MM, Dolz C. Drug-induced hepatitis superimposed on the presence of anti-SLA antibody: a case report. J. Med. Case Reports 2: 25 (2008). *

• European Association for the Study of the Liver. EASL clinical practice guidelines: management of cholestatic liver diseases. J. Hepatol. 51: 237-267 (2009). *

• Everson GT, Shiffman ML, Morgan TR, Hoefs JC, Sterling RK, Wagner DA, Kulig CC, Curto TM, Wright EC; Halt-C Trial Group. The spectrum of hepatic functional impairment in compensated chronic hepatitis C: results from the Hepatitis C Anti-viral Long-term Treatment against Cirrhosis Trial. Aliment. Pharmacol. Ther. 27: 798-809 (2008). *

• Ezaki H, Yoshida Y, Saji Y, Takemura T, Fukushima J, Matsumoto H, Kamada Y, Wada A, Igura T, Kihara S, Funahashi T, Shimomura I, Tamura S, Kiso S, Hayashi N. Delayed liver regeneration after partial hepatectomy in adiponectin knockout mice. Biochem. Biophys. Res. Commun. 378: 68-72 (2009). *

• Fantappie O, Solazzo M, Lasagna N, Platini F, Tessitore L, Mazzanti R. P-Glycoprotein mediates celecoxib-induced apoptosis in multiple drug-resistant cell lines. Cancer Res. 67: 4915-4923 (2007). *

• Farghali H, Masek K. Immunopharmacologic agents in the amelioration of hepatic injuries. Int. J. Immunopharmacol. 20: 125-139 (1998).

• Farias AQ, Goncalves LL, Bittencourt PL, De Melo ES, Abrantes-Lemos CP, Porta G, Nakhle MC, Carrilho FJ, Cancado EL. Applicability of the IAIHG scoring system to the diagnosis of antimitochondrial/anti-M2 seropositive variant form of autoimmune hepatitis. J. Gastroenterol. Hepatol. 21: 887-893 (2006). *

• Faust TW. Recurrent primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis after transplantation. Semin. Liver Dis. 20: 481-495 (2000). *

• Feinstein DL, Spagnolo A, Akar C, Weinberg G, Murphy P, Gavrilyuk V, Russo CD. Receptor-independent actions of PPAR thiazolidinedione agonists: is mitochondrial function the key? Biochem. Pharmacol. 70: 177-188 (2005). *

• Feist E, Brychcy M, Hausdorf G, Hoyer B, Egerer K, Dorner T, Kuckelkorn U, Burmester GR. Anti-proteasome autoantibodies contribute to anti-nuclear antibody patterns on human larynx carcinoma cells. Ann. Rheum. Dis. 66: 5-11 (2007). *

• Feld J, Heathcote E. Epidemiology of autoimmune liver disease. J. Gastroenterol. Hepatol. 18: 1118-1128 (2003). *

• Feld JJ, Meddings J, Heathcote EJ. Abnormal intestinal permeability in primary biliary cirrhosis. Dig. Dis. Sci. 51: 1607-1613 (2006). *

• Fellman V, Lemmela S, Sajantila A, Pihko H, Jarvela I. Screening of BCS1L mutations in severe neonatal disorders suspicious for mitochondrial cause. J. Hum. Genet. 53: 554-558 (2008). *

• Fernandez D, Bonilla E, Mirza N, Niland B, Perl A. Rapamycin reduces disease activity and normalizes T cell activation-induced calcium fluxing in patients with systemic lupus erythematosus. Arthritis Rheum. 54: 2983-2988 (2006). *

• Fernandez D, Bonilla E, Phillips P, Perl A. Signaling abnormalities in systemic lupus erythematosus as potential drug targets. Endocr. Metab. Immune Disord. Drug Targets 6: 305-311 (2006).

• Fernandez O, Fernandez V, Martinez-Cabrera V, Mayorga C, Alonso A, Leon A, Arnal C, Hens M, Luque G, de Ramon E, Caballero A, Leyva L. Multiple sclerosis in Gypsies from southern Spain: prevalence, mitochondrial DNA haplogroups and HLA class II association. Tissue Antigens 71: 426-433 (2008). *

• Fernandez-Checa JC. Alcohol-induced liver disease: when fat and oxidative stress meet. Ann. Hepatol. 2: 69-75 (2003). *

• Fernandez-Cruz E, Bootello A, Blanco MF, Gosalvez M, Segovia de Arana JM. Primary biliary cirrhosis. Study of the action of mitochondrial antibody plus complement on mitochondrial membrane functions. Allergol. Immunopathol. (Madr.) 4: 145-152 (1976).

• Feuchtinger M, Christ S, Preuss B, Dengjel J, Duman S, Stevanovic S, Klein R. Detection of novel non-M2-related antimitochondrial antibodies in patients with anti-M2 negative primary biliary cirrhosis. Gut 58: 983-989 (2009). *

• Feuchtinger M, Dengjel J, Stevanovic S, Klein R. Identification of F1-ATPse as a new target antigen of antimitochondrial antibodies (AMA) in primary biliary cirrhosis (PBC). J. Hepatol. 40 Suppl. 1: 159 (2004).

• Fickert P, Trauner M. When lightning strikes twice: the plot thickens for a dual role of the anion exchanger 2 (AE2/SLC4A2) in the pathogenesis and treatment of primary biliary cirrhosis. J. Hepatol. 50: 633-635 (2009). *

• Fiducia M, Lauretta R, Lunghi R, Kyanvash S, Pallotti S. Hashimoto's thyroiditis and autoimmunity parameters: descriptive study. Minerva Med. 98: 95-99 (2007).

• Filipovic B, Opric M, Milicic A, Paranos S. Primary biliary cirrhosis. Srp. Arh. Celok. Lek. 130: 213-216 (2002).

• Findor JA, Sorda JA, Daruich JR, Manero EF. Familial association in autoimmune liver disease. Medicina (B. Aires) 62: 241-244 (2002).

• Flachs P, Horakova O, Brauner P, Rossmeisl M, Pecina P, Franssen-van Hal N, Ruzickova J, Sponarova J, Drahota Z, Vlcek C, Keijer J, Houstek J, Kopecky J. Polyunsaturated fatty acids of marine origin upregulate mitochondrial biogenesis and induce beta-oxidation in white fat. Diabetologia 48: 2365-2375 (2005). *

• Flachs P, Rossmeisl M, Bryhn M, Kopecky J. Cellular and molecular effects of n-3 polyunsaturated fatty acids on adipose tissue biology and metabolism. Clin. Sci. (Lond.) 116: 1-16 (2009). *

• Flamigni F, Stanic' I, Facchini A, Cetrullo S, Tantini B, Borzì RM, Guarnieri C, Caldarera CM. Polyamine biosynthesis as a target to inhibit apoptosis of non-tumoral cells. Amino Acids 33: 197-202 (2007). *

• Flannery GR, Burroughs AK, Butler P, Chelliah J, Hamilton-Miller J, Brumfitt W, Baum H. Antimitochondrial antibodies in primary biliary cirrhosis recognize both specific peptides and shared epitopes of the M2 family of antigens. Hepatology 10: 370-374 (1989).

• Fleischer RD, Lok AS. Myopathy and neuropathy associated with nucleos(t)ide analog therapy for hepatitis B. J. Hepatol. 51: 787-791 (2009).

• Flisiak R, Pelszynska M, Prokopowicz D, Rogalska M, Grygoruk U. High concentration of antimitochondrial antibodies predicts progressive primary biliary cirrhosis. World J. Gastroenterol. 11: 5706-5709 (2005). *

• Flisiak R, Wiercinska-Drapalo A, Prokopowicz D. Antibodies against M2-antigen in differential diagnosis of primary biliary cirrhosis. Pol. Merkuriusz. Lek. 8: 373-375 (2000).

• Floreani A, Baragiotta A, Leone MG, Baldo V, Naccarato R. Primary biliary cirrhosis and hepatitis C virus infection. Am. J. Gastroenterol. 98: 2757-2762 (2003). *

• Floreani A, Betterle C, Baragiotta A, Martini S, Venturi C, Basso D, Pittoni M, Chiarelli S, Sategna Guidetti C. Prevalence of coeliac disease in primary biliary cirrhosis and of antimitochondrial antibodies in adult coeliac disease patients in Italy. Dig. Liver Dis. 34: 258-261 (2002).

• Foger N, Rangell L, Danilenko DM, Chan AC. Requirement for coronin 1 in T lymphocyte trafficking and cellular homeostasis. Science 313: 839-842 (2006). *

• Fontana RJ. Side effects of long-term oral antiviral therapy for hepatitis B. Hepatology 49: S185-S195 (2009).

• Forns X, Sanchez Tapias JM, Pares A, Llovet JM, Bruix J, Rodes J. Expected developments in hepatology. Best Pract. Res. Clin. Gastroenterol. 16: 957-970 (2002). *

• Foroncewicz B, Mucha K, Paczek L, Oldakowska-Jedynak U, Gornicka B, Zieniewicz K, Nyckowski P, Krawczyk M. Anti-CD25 and tacrolimus therapy may not prevent early primary biliary cirrhosis recurrence after liver transplantation: two case reports. Transplant. Proc. 35: 2310-2312 (2003). *

• Forte M, Gold BG, Marracci G, Chaudhary P, Basso E, Johnsen D, Yu X, Fowlkes J, Bernardi P, Bourdette D. Cyclophilin D inactivation protects axons in experimental autoimmune encephalomyelitis, an animal model of multiple sclerosis. Proc. Natl. Acad. Sci. U.S.A. 104: 7558-7563 (2007). *

• Fortun PJ, Hawkey CJ. Nonsteroidal antiinflammatory drugs and the small intestine. Curr. Opin. Gastroenterol. 21: 169-175 (2005).

• Foschini MP, Macchia S, Losi L, Dei Tos AP, Pasquinelli G, Di Tommaso L, Del Duca S, Roncaroli F, Dal Monte PR. Identification of mitochondria in liver biopsies. A study by immunohistochemistry, immunogold and Western blot analysis. Virchows. Arch. 433: 267-273 (1998). *

• Foteinos G, Xu Q. Immune-mediated mechanisms of endothelial damage in atherosclerosis. Autoimmunity 42: 627-633 (2009).

• Franco J, Saeian K. Biliary tract inflammatory disorders: primary sclerosing cholangitis and primary biliary cirrhosis. Curr. Gastroenterol. Rep. 1: 95-101 (1999).

• Frazer IH, Mackay IR, Jordan TW, Whittingham S, Marzuki S. Reactivity of anti-mitochondrial autoantibodies in primary biliary cirrhosis: definition of two novel mitochondrial polypeptide autoantigens. J. Immunol. 135: 1739-1745 (1985).

• Fregeau DR, Davis PA, Danner DJ, Ansari A, Coppel RL, Dickson ER, Gershwin ME. Antimitochondrial antibodies of primary biliary cirrhosis recognize dihydrolipoamide acyltransferase and inhibit enzyme function of the branched chain alpha-ketoacid dehydrogenase complex. J. Immunol. 142: 3815-3820 (1989).

• Fregeau DR, Leung PS, Coppel RL, McNeilage LJ, Medsger TA Jr, Gershwin ME. Autoantibodies to mitochondria in systemic sclerosis. Frequency and characterization using recombinant cloned autoantigen. Arthritis Rheum. 31: 386-392 (1988).

• Fregeau DR, Prindiville T, Coppel RL, Kaplan M, Dickson ER, Gershwin ME. Inhibition of alpha-ketoglutarate dehydrogenase activity by a distinct population of autoantibodies recognizing dihydrolipoamide succinyltransferase in primary biliary cirrhosis. Hepatology 11: 975-981 (1990).

• Fregeau DR, Roche TE, Davis PA, Coppel R, Gershwin ME. Primary biliary cirrhosis. Inhibition of pyruvate dehydrogenase complex activity by autoantibodies specific for E1 alpha, a non-lipoic acid containing mitochondrial enzyme. J. Immunol. 144: 1671-1676 (1990).

• French MA, Bernstein RM. Immunoglobulin G subclass distribution of autoantibodies in systemic sclerosis, primary biliary cirrhosis, and overlap syndromes. Ann. Rheum. Dis. 46: 436-440 (1987). *

• Frezza M, Terpin MM, Peri A. S-Adenosyl-L-methionine (SAMe) and its use in hepatology. Minerva Gastroenterol. Dietol. 38: 145-151 (1992).

• Fritah A, Steel JH, Nichol D, Parker N, Williams S, Price A, Strauss L, Ryder TA, Mobberley MA, Poutanen M, Parker M, White A. Elevated expression of the metabolic regulator RIP140 results in cardiac hypertrophy and impaired cardiac function. Cardiovasc. Res. Jan 18 [Epub ahead of print] (2010). *

• Froy O, Chapnik N, Miskin R. Long-lived alphaMUPA transgenic mice exhibit pronounced circadian rhythms. Am. J. Physiol. Endocrinol. Metab. 291: E1017-E1024 (2006). *

• Fruchart JC, Duriez P, Staels B. Peroxisome proliferator-activated receptor-alpha activators regulate genes governing lipoprotein metabolism, vascular inflammation and atherosclerosis. Curr. Opin. Lipidol. 10: 245-257 (1999).

• Fuenzalida K, Quintanilla R, Ramos P, Piderit D, Fuentealba RA, Martinez GR, Inestrosa NC, Bronfman M. Peroxisome proliferator-activated receptor gamma up-regulates the Bcl-2 anti-apoptotic protein in neurons and induces mitochondrial stabilization and protection against oxidative stress and apoptosis. J. Biol. Chem. 282: 37006-37015 (2007). *

• Fujikura S, Davis PA, Prindiville T, Leung P, Fox RI, Gershwin ME. Sjogren's syndrome and primary biliary cirrhosis: presence of autoantibodies to purified mitochondrial 2-oxo acid dehydrogenases. J. Rheumatol. 17: 1453-1457 (1990).

• Fujimoto M, Sato S, Ihn H, Tamaki T, Kikuchi K, Soma Y, Tamaki K. Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma. Clin. Exp. Immunol. 105: 297-301 (1996). *

• Fujioka S, Yamamoto K, Okamoto R, Miyake M, Ujike K, Shimada N, Terada R, Miyake Y, Nakajima H, Piao CY, Iwasaki Y, Tanimizu M, Tsuji T. Laparoscopic features of primary biliary cirrhosis in AMA-positive and AMA-negative patients. Endoscopy 34: 318-321 (2002).

• Fujisawa A, Kambe N, Saito M, Nishikomori R, Tanizaki H, Kanazawa N, Adachi S, Heike T, Sagara J, Suda T, Nakahata T, Miyachi Y. Disease-associated mutations in CIAS1 induce cathepsin B-dependent rapid cell death of human THP-1 monocytic cells. Blood 109: 2903-2911 (2007). *

• Fukushima K, Ueno Y, Shimosegawa T. Treatment of primary biliary cirrhosis: a new challenge? Hepatol. Res. 40: 61-68 (2010). *

• Fukushima N, Ikematsu H, Nakamura M, Matsui M, Shimoda S, Hayashida K, Niho Y, Koike K, Gershwin ME, Ishibashi H. Nucleotide variations amongst V(H) genes of AMA-producing B cell clones in primary biliary cirrhosis. J. Autoimmun. 14: 247-257 (2000). *

• Fukushima N, Nakamura M, Matsui M, Ikematsu H, Koike K, Ishibashi H, Hayashida K, Niho Y. Establishment and structural analysis of human mAb to the E2 component of the 2-oxoglutarate dehydrogenase complex generated from a patient with primary biliary cirrhosis. Int. Immunol. 7: 1047-1055 (1995).

• Fukushima N, Nalbandian G, Van De Water J, White K, Ansari AA, Leung P, Kenny T, Kamita SG, Hammock BD, Coppel RL, Stevenson F, Ishibashi H, Gershwin ME. Characterization of recombinant monoclonal IgA anti-PDC-E2 autoantibodies derived from patients with PBC. Hepatology 36: 1383-1392 (2002). *

• Fulop P, Derdak Z, Sheets A, Sabo E, Berthiaume EP, Resnick MB, Wands JR, Paragh G, Baffy G. Lack of UCP2 reduces fas-mediated liver injury in ob/ob mice and reveals importance of cell-specific UCP2 expression. Hepatology 44: 592-601 (2006). *

• Furnsinn C, Willson TM, Brunmair B. Peroxisome proliferator-activated receptor-delta, a regulator of oxidative capacity, fuel switching and cholesterol transport. Diabetologia 50: 8-17 (2007). *

• Furuya T, Tateishi M, Nishinarita M, Kashiwazaki S. Primary biliary cirrhosis in patients with Sjogren's syndrome. Nippon Rinsho 53: 2536-2539 (1995).

• Fusconi M, Baum H, Caselli A, Cassani F, Ballardini G, Lenzi M, Volta U, Zauli D, Bianchi FB. Demonstration of peptide-specific and cross-reactive epitopes in proteins reacting with antimitochondrial antibodies of primary biliary cirrhosis. J. Hepatol. 15: 162-169 (1992).

• Fusconi M, Ghadiminejad I, Bianchi FB, Baum H, Bottazzo GF, Pisi E. Heterogeneity of antimitochondrial antibodies with the M2-M4 pattern by immunofluorescence as assessed by Western immunoblotting and enzyme linked immunosorbent assay. Gut 29: 440-447 (1988). *

• Fussey SP, Ali ST, Guest JR, James OF, Bassendine MF, Yeaman SJ. Reactivity of primary biliary cirrhosis sera with Escherichia coli dihydrolipoamide acetyltransferase (E2p): characterization of the main immunogenic region. Proc. Natl. Acad. Sci. U.S.A. 87: 3987-3991 (1990). *

• Fussey SP, Bassendine MF, Fittes D, Turner IB, James OF, Yeaman SJ. The E1 alpha and beta subunits of the pyruvate dehydrogenase complex are M2'd' and M2'e' autoantigens in primary biliary cirrhosis. Clin. Sci. (Lond.) 77: 365-368 (1989). *

• Fussey SP, Guest JR, James OF, Bassendine MF, Yeaman SJ. Identification and analysis of the major M2 autoantigens in primary biliary cirrhosis. Proc. Natl. Acad. Sci. U.S.A. 85: 8654-8658 (1988). *

• Fussey SP, West SM, Lindsay JG, Ragan CI, James OF, Bassendine MF, Yeaman SJ. Clarification of the identity of the major M2 autoantigen in primary biliary cirrhosis. Clin. Sci. (Lond.) 80: 451-455 (1991).

• Gabeta S, Norman GL, Liaskos C, Papamichalis PA, Zografos T, Garagounis A, Rigopoulou EI, Dalekos GN. Diagnostic relevance and clinical significance of the new enhanced performance M2 (MIT3) ELISA for the detection of IgA and IgG antimitochondrial antibodies in primary biliary cirrhosis. J. Clin. Immunol. 27: 378-387 (2007). *

• Galambos JT, Brooks WS Jr. Atypical biliary cirrhosis - or sclerosing cholangitis. J. Clin. Gastroenterol. 2: 43-52 (1980).

• Galperin C, Gershwin ME. Immunopathology of primary biliary cirrhosis. Baillieres Clin. Gastroenterol. 10: 461-481 (1996).

• Garcia Villarreal L, Zozaya JM, Macias E, Garcia Gonzalez N, Quiroga J, Conchillo F, Prieto J. The treatment of primary biliary cirrhosis with ursodeoxycholic acid. The short- and median-term results and their relation to the study of the disease. Rev. Esp. Enferm. Dig. 80: 311-315 (1991).

• Garcia-Ruiz C, Fernandez-Checa JC. Redox regulation of hepatocyte apoptosis. J. Gastroenterol. Hepatol. 22 Suppl. 1: S38-S42 (2007). *

• Garcia-Ruiz I, Rodriguez-Juan C, Diaz-Sanjuan T, Del Hoyo P, Colina F, Munoz-Yague T, Solis-Herruzo JA. Uric acid and anti-TNF antibody improve mitochondrial dysfunction in ob/ob mice. Hepatology 44: 581-591 (2006). *

• Gatselis NK, Stefos A, Gioti C, Rigopoulou EI, Dalekos GN. Primary biliary cirrhosis and Henoch-Schonlein purpura: report of two cases and review of the literature. Liver Int. 27: 280-283 (2007). *

• Geetha A, Parameswari S. Effect of ursodeoxycholic acid on hydrogen peroxide induced lipid peroxidation in sheep liver mitochondria. Indian J. Physiol. Pharmacol. 46: 343-348 (2002).

• Gerber MA, Thung SN. Ultrastructural localization of mitochondrial and ribosomal antigens by peroxidase-labeled human antibodies. Lab. Invest. 39: 101-105 (1978).

• German OL, Insua MF, Gentili C, Rotstein NP, Politi LE. Docosahexaenoic acid prevents apoptosis of retina photoreceptors by activating the ERK/MAPK pathway. J. Neurochem. 98: 1507-1520 (2006). *

• Gershwin ME. Primary biliary cirrhosis: from induction through destruction. Falk Symposium 142: GASTROENTEROLOGY WEEK FREIBURG 2004 (Part I) AUTOIMMUNE LIVER DISEASE, Freiburg (Germany), October 12 - 13, 2004, pp. 39-40 (2004). *

• Gershwin ME. The immunopathology of primary biliary cirrhosis: unraveling the mystery of etiology. Falk Symposium 146: GUT-LIVER INTERACTIONS: BASIC AND CLINICAL CONCEPTS, Innsbruck (Austria), March 11-12, 2005, p. 61 (2005). *

• Gershwin ME, Ansari AA, Mackay IR, Nakanuma Y, Nishio A, Rowley MJ, Coppel RL. Primary biliary cirrhosis: an orchestrated immune response against epithelial cells. Immunol. Rev. 174: 210-225 (2000). *

• Gershwin ME, Leung PS, Li H, Seldin MF. Primary biliary cirrhosis and autoimmunity: evaluating the genetic risk. Isr. Med. Assoc. J. 2 Suppl: 7-10 (2000).

• Gershwin ME, Mackay IR. New knowledge in primary biliary cirrhosis. Hosp. Pract. (Off. Ed.) 30: 29-36 (1995).

• Gershwin ME, Mackay IR. The causes of primary biliary cirrhosis: convenient and inconvenient truths. Hepatology 47: 737-745 (2008). *

• Gershwin ME, Mackay IR. Primary biliary cirrhosis: paradigm or paradox for autoimmunity. Gastroenterology 100: 822-833 (1991).

• Gershwin ME, Mackay IR, Sturgess A, Coppel RL. Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis. J. Immunol. 138: 3525-3531 (1987).

• Gershwin ME, Rowley M, Davis PA, Leung P, Coppel R, Mackay IR. Molecular biology of the 2-oxo-acid dehydrogenase complexes and anti-mitochondrial antibodies. Prog. Liver Dis. 10: 47-61 (1992).

• Gershwin ME, Van de Water J, Leung P, Ansari AA, Coppel RL. The pathobiology and the cause of primary biliary cirrhosis. Falk Symposium 135. Immmunological diseases of liver and gut. Prague (Czech Republic). p. 36-37. September 12 - 13 (2003). *

• Gershwin ME, Van de WaterJ, Leung P, Ansari AA, Coppel RL. Primary biliary cirrhosis: from induction through destruction. Falk Symposium 136. XII FALK LIVER WEEK 2003 (Part I) In Honour of Hans Popper’s 100th Birthday. Cholestatic Liver Diseases: Therapeutical Options and Perspectives. Freiburg (Germany). p. 18. October 15-16 (2003). *

• Ghadiminejad I, Baum H. Discrimination between M2 and M4 antimitochondrial antibodies in primary biliary cirrhosis. Biosci. Rep. 6: 1049-1056 (1986).

• Ghadiminejad I, Baum H. Evidence for the cell-surface localization of antigens cross-reacting with the "mitochondrial antibodies" of primary biliary cirrhosis. Hepatology 7: 743-749 (1987).

• Ghadiminejad I, Baum H. Reaction pattern of mitochondrial antibodies of primary biliary cirrhosis (PBC) is species specific but not organ specific. J. Bioenerg. Biomembr. 19: 239-253 (1987).

• Gheorghe L, Iacob S, Bolog N, Stoicescu A, Parvulescu I, Popescu I. Overlap syndrome between autoimmune hepatitis and primary biliary cirrhosis complicated by hepatocellular carcinoma. Case report. Rom. J. Gastroenterol. 13: 33-38 (2004).

• Ghezzi A, Zaffaroni M. Neurological manifestations of gastrointestinal disorders, with particular reference to the differential diagnosis of multiple sclerosis. Neurol. Sci. 22 Suppl. 2: S117-S122 (2001).

• Ghezzi D, Goffrini P, Uziel G, Horvath R, Klopstock T, Lochmuller H, D'Adamo P, Gasparini P, Strom TM, Prokisch H, Invernizzi F, Ferrero I, Zeviani M. SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. Nat. Genet. 41: 654-656 (2009). *

• Ghonaim M, Al-Ghamdi A, El-Bana H, Bakr A, Ghoneim E, El-Edel R, Hassona M, Shoeib S, Allam H. Autoantibodies in chronic liver disease. Egypt. J. Immunol. 12: 101-111 (2005).

• Ghosh A, Sil PC. Protection of acetaminophen induced mitochondrial dysfunctions and hepatic necrosis via Akt-NF-kappaB pathway: role of a novel plant protein. Chem. Biol. Interact. 177: 96-106 (2009). *

• Gilbrud B, Weiss P, Bakimer R, Shoenfeld Y. Identification and characterization of antimitochondrial autoantibodies in sera of patients with monoclonal gammopathies. Immunol. Lett. 45: 163-166 (1995).

• Gilburd B, Ziporen L, Zharhary D, Blank M, Zurgil N, Scheinberg MA, Guedes LH, Gershwin ME, Shoenfeld Y. Antimitochondrial (pyruvate dehydrogenase) antibodies in leprosy. J. Clin. Immunol. 14: 14-19 (1994).

• Giorgini A, Selmi C, Invernizzi P, Podda M, Zuin M, Gershwin ME. Primary biliary cirrhosis: solving the enigma. Ann. N. Y. Acad. Sci. 1051: 185-193 (2005). *

• Gisbert JP, Jones EA, Pajares JM, Moreno-Otero R. Review article: is there an optimal therapeutic regimen for antimitochondrial antibody-negative primary biliary cirrhosis (autoimmune cholangitis)? Aliment. Pharmacol. Ther. 17: 17-27 (2003). *

• Gockel HR, Lugering A, Heidemann J, Schmidt M, Domschke W, Kucharzik T, Lugering N. Thalidomide induces apoptosis in human monocytes by using a cytochrome c-dependent pathway. J. Immunol. 172: 5103-5109 (2004). *

• Gogos CA, Nikolopoulou V, Zolota V, Siampi V, Vagenakis A. Autoimmune cholangitis in a patient with celiac disease: a case report and review of the literature. J. Hepatol. 30: 321-324 (1999). *

• Golovnova EV, Il'chenko LIu, Tsaregorodtseva TM, Serova TI, Gudkova RV, Shepeleva SD, Tkachev VD. Primary biliary cirrhosis: 12-year experience in observation. Ter. Arkh. 75: 26-30 (2003).

• Gong P, Cederbaum AI. Nrf2 is increased by CYP2E1 in rodent liver and HepG2 cells and protects against oxidative stress caused by CYP2E1. Hepatology 43: 144-153 (2006). *

• Gong Y, Gluud C. Colchicine for primary biliary cirrhosis: a Cochrane hepato-biliary group systematic review of randomized clinical trials. Am. J. Gastroenterol. 100: 1-10 (2005). *

• Gonzalez M, Ribalta J, Reyes H, Klinger J, Maggiolo P, Varleta P, Hernandez I, Delgado M, Donoso S. Primary biliary cirrhosis. The clinical experience in 31 patients. Rev. Med. Chil. 118: 1350-1354 (1990).

• Gonzalez-Huezo MS, Villela LM, Zepeda-Florencio Mdel C, Carrillo-Ponce CS, Mondragon-Sanchez RJ. Nodular regenerative hyperplasia associated to aplastic anemia: a case report and literature review. Ann. Hepatol. 5: 166-169 (2006). *

• Goodman ZD, McNally PR, Davis DR, Ishak KG. Autoimmune cholangitis: a variant of primary biliary cirrhosis. Clinicopathologic and serologic correlations in 200 cases. Dig. Dis. Sci. 40: 1232-1242 (1995).

• Goring HD, Panzner M, Lakotta W, Ziemer A. Coincidence of scleroderma and primary biliary cirrhosis. Results of a systematic study of a dermatologic patient sample. Hautarzt. 49: 361-366 (1998).

• Gouw AS, Haagsma EB, Manns M, Klompmaker IJ, Slooff MJ, Gerber MA. Is there recurrence of primary biliary cirrhosis after liver transplantation? A clinicopathologic study in long-term survivors. J. Hepatol. 20: 500-507 (1994).

• Grabacka M, Reiss K. Anticancer properties of PPARalpha - effects on cellular metabolism and inflammation. PPAR Res. 2008: 930705 (2008). *

• Graf D, Kurz AK, Reinehr R, Fischer R, Kircheis G, Haussinger D. Prevention of bile acid-induced apoptosis by betaine in rat liver. Hepatology 36: 829-839 (2002). *

• Granito A, Muratori P, Muratori L, Pappas G, Cassani F, Worthington J, Ferri S, Quarneti C, Cipriano V, DE Molo C, Lenzi M, Chapman RW, Bianchi FB. Antibodies to SS-A/Ro-52kD and centromere in autoimmune liver disease: a clue to diagnosis and prognosis of primary biliary cirrhosis. Aliment. Pharmacol. Ther. 26: 831-838 (2007). *

• Granito A, Muratori P, Muratori L, Pappas G, Cassani F, Worthington J, Guidi M, Ferri S, DE Molo C, Lenzi M, Chapman RW, Bianchi FB. Antinuclear antibodies giving the 'multiple nuclear dots' or the 'rim-like/membranous' patterns: diagnostic accuracy for primary biliary cirrhosis. Aliment. Pharmacol. Ther. 24: 1575-1583 (2006). *

• Granito A, Yang WH, Muratori L, Lim MJ, Nakajima A, Ferri S, Pappas G, Quarneti C, Bianchi FB, Bloch DB, Muratori P. PML nuclear body component Sp140 is a novel autoantigen in primary biliary cirrhosis. Am. J. Gastroenterol. 105: 125-131 (2010). *

• Grattagliano I, Bonfrate L, Diogo CV, Wang HH, Wang DQ, Portincasa P. Biochemical mechanisms in drug-induced liver injury: certainties and doubts. World J. Gastroenterol. 15: 4865-4876 (2009). *

• Gregorio GV, Portmann B, Mowat AP, Vergani D, Mieli-Vergani G. A 12-year-old girl with antimitochondrial antibody-positive autoimmune hepatitis. J. Hepatol. 27: 751-754 (1997). *

• Grimbert S, Johanet C, Bendjaballah F, Homberg JC, Poupon R, Beaugrand M. Antimitochondrial antibodies in patients with chronic hepatitis C. Liver 16: 161-165 (1996).

• Gruber R, Felber E, Pape GR, Hochtlen-Vollmar W, Riethmuller G. Detection of autoantibodies against M2, LKM-1, and SLA in liver diseases by standardized uniform ELISA-techniques. J. Clin. Lab. Anal. 8: 284-292 (1994).

• Grunewald RW, Fiedler GM, Stockmann F, Schauer A, Muller GA. Unusual diagnosis in recurrent arthritis, erythema nodosum and arrhythmia. Dtsch. Med. Wochenschr. 122: 1516-1520 (1997).

• Guedes Barbosa LS, Gilbrut B, Shoenfeld Y, Scheinberg MA. Autoantibodies in leprosy sera. Clin. Rheumatol. 15: 26-28 (1996).

• Gumpricht E, Dahl R, Devereaux MW, Sokol RJ. Licorice compounds, glycyrrhizin and 18beta -glycyrrhetinic acid, are potent modulators of bile acid-induced cytotoxicity in rat hepatocytes. J. Biol. Chem. 280: 10556-10563 (2005). *

• Gumpricht E, Dahl R, Devereaux MW, Sokol RJ. Beta-carotene prevents bile acid-induced cytotoxicity in the rat hepatocyte: evidence for an antioxidant and anti-apoptotic role of beta-carotene in vitro. Pediatr. Res. 55: 814-821 (2004). *

• Gundling F, Rohrbach H, Schepp W. Cholestatic hepatitis in situs inversus abdominalis. Schweiz. Rundsch. Med. Prax. 96: 1029-1033 (2007).

• Guo CJ, Pan Q, Cheng T, Jiang B, Chen GY, Li DG. Changes in microRNAs associated with hepatic stellate cell activation status identify signaling pathways. FEBS J. 276: 5163-5176 (2009). *

• Gupta R, Agarwal SR, Jain M, Malhotra V, Sarin SK. Autoimmune hepatitis in the Indian subcontinent: 7 years experience. J. Gastroenterol. Hepatol. 16: 1144-1148 (2001). *

• Gupta RC. Specificities and characteristics of mitochondrial protein antigens assessed by enzyme linked immunosorbent assay for anti-mitochondrial antibodies. Relationship to immunofluorescent and precipitating antibodies. Clin. Exp. Immunol. 59: 604-612 (1985). *

• Gupta RC, Dickson ER, McDuffie FC, Baggenstoss AH. Circulating IgG complexes in primary biliary cirrhosis. A serial study in forty patients followed for two years. Clin. Exp. Immunol. 34: 19-27 (1978). *

• Gupta RC, Seibold JR, Krishnan MR, Steigerwald JC. Precipitating autoantibodies to mitochondrial proteins in progressive systemic sclerosis. Clin. Exp. Immunol. 58: 68-76 (1984). *

• Gupta RC, Tan EM. Isolation of circulating immune complexes by conglutinin and separation of antigen from dissociated complexes by immobilized protein A. Clin. Exp. Immunol. 46: 9-19 (1981). *

• Gut J, Christen U, Huwyler J. Mechanisms of halothane toxicity: novel insights. Pharmacol. Ther. 58: 133-155 (1993).

• Gutierrez Macia MM, Mateo García MG, Vidal Marsal F, Domingo Pedrol P. Toxicogenetics of antiretroviral treatment (1): lipodystrophy, metabolic perturbations and atherosclerosis. Enferm. Infecc. Microbiol. Clin. 26 Suppl. 6: 18-23 (2008).

• Guy J. Optic nerve degeneration in experimental autoimmune encephalomyelitis. Ophthalmic Res. 40: 212-216 (2008).

• Gvozdjakova A, Kucharska J, Bartkovjakova M, Gazdikova K, Gazdik FE. Coenzyme Q10 supplementation reduces corticosteroids dosage in patients with bronchial asthma. Biofactors 25: 235-240 (2005).

• Gyamfi MA, Kocsis MG, He L, Dai G, Mendy AJ, Wan YJ. The role of retinoid X receptor alpha in regulating alcohol metabolism. J. Pharmacol. Exp. Ther. 319: 360-368 (2006). *

• Haagsma EB, Manns M, Klein R, Grond J, Huizenga JR, Slooff MJ, Meyer zum Buschenfelde KH, Berg PA, Gips CH. Subtypes of antimitochondrial antibodies in primary biliary cirrhosis before and after orthotopic liver transplantation. Hepatology 7: 129-133 (1987).

• Haberhauer G, Wandner G, Machacek E, Broll H. CRST syndrome and primary biliary cirrhosis. Z. Rheumatol. 46: 341-343 (1987).

• Haitina T, Lindblom J, Renstrom T, Fredriksson R. Fourteen novel human members of mitochondrial solute carrier family 25 (SLC25) widely expressed in the central nervous system. Genomics 88: 779-790 (2006). *

• Hall S, Axelsen PH, Larson DE, Bunch TW. Systemic lupus erythematosus developing in patients with primary biliary cirrhosis. Ann. Intern. Med. 100: 388-389 (1984).

• Hama K, Miwa H, Nishino I, Nonaka I, Kondo T. Inclusion body myositis associated with chronic thyroiditis, Sjogren's syndrome and autoimmune cholangitis. No To Shinkei 56: 503-507 (2004).

• Hamada M, Tokumoto Y, Furukawa S, Minami H, Hiasa Y, Michitaka K, Horiike N, Onji M. Hepatopulmonary syndrome in a patient with primary biliary cirrhosis. Intern. Med. 43: 458-460 (2004). *

• Han Y, Zhang W, Chen J, Zhou H, Wang L. Clinical and pathological study of autoimmune hepatopathy. Zhonghua Gan Zang Bing Za Zhi 9: 82S-84S (2001).

• Hanson JC, Bostick MK, Campe CB, Kodali P, Lee G, Yan J, Maher JJ. Transgenic overexpression of interleukin-8 in mouse liver protects against galactosamine/endotoxin toxicity. J. Hepatol. 44: 359-367 (2006). *

• Harada K, Sudo Y, Kono N, Ozaki S, Tsuneyama K, Gershwin ME, Nakanuma Y. In situ nucleic acid detection of PDC-E2, BCOADC-E2, OGDC-E2, PDC-E1alpha, BCOADC-E1alpha, OGDC-E1, and the E3 binding protein (protein X) in primary biliary cirrhosis. Hepatology 30: 36-45 (1999). *

• Harada K, Van de Water J, Leung PS, Coppel RL, Nakanuma Y, Gershwin ME. In situ nucleic acid hybridization of pyruvate dehydrogenase complex-E2 in primary biliary cirrhosis: pyruvate dehydrogenase complex-E2 messenger RNA is expressed in hepatocytes but not in biliary epithelium. Hepatology 25: 27-32 (1997). *

• Haratake J, Kasai T, Makino H. Diffuse mucosal carcinoma of intrahepatic and extrahepatic bile ducts including gallbladder. Pathol. Int. 52: 784-788 (2002). *

• Hardeland R. Melatonin: signaling mechanisms of a pleiotropic agent. Biofactors 35: 183-192 (2009).

• Harhaji L, Isakovic A, Vucicevic L, Janjetovic K, Misirkic M, Markovic Z, Todorovic-Markovic B, Nikolic N, Vranjes-Djuric S, Nikolic Z, Trajkovic V. Modulation of tumor necrosis factor-mediated cell death by fullerenes. Pharm. Res. 25: 1365-1376 (2008). *

• Hartel M, di Mola FF, Selvaggi F, Mascetta G, Wente MN, Felix K, Giese NA, Hinz U, Di Sebastiano P, Buchler MW, Friess H. Vanilloids in pancreatic cancer: potential for chemotherapy and pain management. Gut 55: 519-528 (2006). *

• Haruta I, Kikuchi K, Hashimoto E, Nakamura M, Miyakawa H, Hirota K, Shibata N, Kato H, Arimura Y, Kato Y, Uchiyama T, Nagamune H, Kobayashi M, Miyake Y, Shiratori K, Yagi J. Long-term bacterial exposure can trigger nonsuppurative destructive cholangitis associated with multifocal epithelial inflammation. Lab. Invest. Feb 8 [Epub ahead of print] (2010). *

• Hashimoto E, Shimada M, Noguchi S, Taniai M, Tokushige K, Hayashi N, Takasaki K, Fuchinoue S, Ludwig J. Disease recurrence after living liver transplantation for primary biliary cirrhosis: a clinical and histological follow-up study. Liver Transpl. 7: 588-595 (2001). *

• Hashimoto E, Taniai M, Yatsuji S, Tokushige K, Shiratori K, Yamamoto M, Fuchinoue S. Long-term clinical outcome of living-donor liver transplantation for primary biliary cirrhosis. Hepatol. Res. 37 Suppl. 3: S455-S461 (2007). *

• Hashimoto T, Cook WS, Qi C, Yeldandi AV, Reddy JK, Rao MS. Defect in peroxisome proliferator-activated receptor alpha-inducible fatty acid oxidation determines the severity of hepatic steatosis in response to fasting. J. Biol. Chem. 275: 28918-28928 (2000). *

• Hasselstrom K, Nilsson LA, Wallerstedt S. The predictive value of antimitochondrial antibodies in establishing the diagnosis of primary biliary cirrhosis. Scand. J. Gastroenterol. 23: 103-106 (1988).

• Hatano E, Tanaka A, Kanazawa A, Tsuyuki S, Tsunekawa S, Iwata S, Takahashi R, Chance B, Yamaoka Y. Inhibition of tumor necrosis factor-induced apoptosis in transgenic mouse liver expressing creatine kinase. Liver Int. 24: 384-393 (2004). *

• Hatzis GS, Fragoulis GE, Karatzaferis A, Delladetsima I, Barbatis C, Moutsopoulos HM. Prevalence and longterm course of primary biliary cirrhosis in primary Sjogren's syndrome. J. Rheumatol. 35: 2012-2016 (2008). *

• Hayase Y, Iwasaki S, Akisawa N, Saibara T, Kadokawa Y, Omagari K, Maeda T, Onishi S. Similar anti-mitochondrial antibody reactivity profiles in familial primary biliary cirrhosis. Hepatol. Res. 33: 33-38 (2005).

• Hays SB, Camisa C. Rendu-Osler-Weber-like telangiectasia associated with primary biliary cirrhosis. Cutis. 35: 152-153 (1985).

• Hazama H, Omagari K, Masuda J, Kinoshita H, Ohba K, Sakimura K, Matsuo I, Isomoto H, Murase K, Murata I, Kohno S. Serial changes in enzyme inhibitory antibody to pyruvate dehydrogenase complex during the course of primary biliary cirrhosis. J. Clin. Lab. Anal. 14: 208-213 (2000). *

• Hazama H, Omagari K, Masuda J, Ohba K, Kinoshita H, Matsuo I, Isomoto H, Mizuta Y, Murase K, Murata I, Kohno S. Automated enzymatic mitochondrial antibody assay for the diagnosis of primary biliary cirrhosis: applications of a routine diagnostic tool for the detection of antimitochondrial antibodies. J. Gastroenterol. Hepatol. 17: 316-323 (2002). *

• Heathcote EJ. Autoimmune cholangitis. Clin. Liver Dis. 2: 303-311, viii-ix (1998).

• Heathcote EJ. Management of primary biliary cirrhosis. The American Association for the Study of Liver Diseases practice guidelines. Hepatology 31: 1005-1013 (2000). *

• Heathcote EJ. Primary biliary cirrhosis: historical perspective. Clin. Liver Dis. 7: 735-740 (2003).

• Heathcote EJ. Primary biliary cirrhosis. Clin. Liver Dis. 7: xi-xii (2003). *

• Heathcote EJ, Cauch-Dudek K, Walker V, Bailey RJ, Blendis LM, Ghent CN, Michieletti P, Minuk GY, Pappas SC, Scully LJ, et al. The Canadian Multicenter Double-blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatology 19: 1149-1156 (1994).

• Heathcote J. Overlap syndromes - outlier syndromes and changing diagnoses. Falk Symposium 136. XII FALK LIVER WEEK 2003 (Part I) In Honour of Hans Popper’s 100th Birthday. Cholestatic Liver Diseases: Therapeutical Options and Perspectives. Freiburg (Germany). p. 39-41. October 15-16 (2003). *

• Heathcote J. Primary biliary cirrhosis with features of autoimmune hepatitis. Curr. Gastroenterol. Rep. 3: 60-64 (2001).

• Heathcote J. Update on primary biliary cirrhosis. Can. J. Gastroenterol. 14: 43-48 (2000). *

• Heininger K. A unifying hypothesis of Alzheimer's disease. III. Risk factors. Hum. Psychopharmacol. 15: 1-70 (2000).

• Hendrick AM, Mitchison HC, Bird AG, James OF. Paraproteins in primary biliary cirrhosis. Q. J. Med. 60: 681-684 (1986).

• Herrema H, Derks TG, van Dijk TH, Bloks VW, Gerding A, Havinga R, Tietge UJ, Muller M, Smit GP, Kuipers F, Reijngoud DJ. Disturbed hepatic carbohydrate management during high metabolic demand in medium-chain acyl-CoA dehydrogenase (MCAD)-deficient mice. Hepatology 47: 1894-1904 (2008). *

• Herrine SK. Drug-induced liver disease. 54th Annual Meeting of the American Association for the Study of Liver Diseases (2003).

• Heseltine L, Turner IB, Fussey SP, Kelly PJ, James OF, Yeaman SJ, Bassendine MF. Primary biliary cirrhosis. Quantitation of autoantibodies to purified mitochondrial enzymes and correlation with disease progression. Gastroenterology 99: 1786-1792 (1990).

• Heyman SN, Spectre G, Aamar S, Rubinger D, Pappo O, Ackerman Z. Autoimmune cholangiopathy associated with systemic lupus erythematosus. Liver 22: 102-106 (2002).

• Higami Y, Tsuchiya T, Chiba T, Yamaza H, Muraoka I, Hirose M, Komatsu T, Shimokawa I. Hepatic gene expression profile of lipid metabolism in rats: impact of caloric restriction and growth hormone/insulin-like growth factor-1 suppression. J. Gerontol. A. Biol. Sci. Med. Sci. 61: 1099-1110 (2006).

• Higuchi H, Miyoshi H, Bronk SF, Zhang H, Dean N, Gores GJ. Bid antisense attenuates bile acid-induced apoptosis and cholestatic liver injury. J. Pharmacol. Exp. Ther. 299: 866-873 (2001). *

• Hikita H, Takehara T, Kodama T, Shimizu S, Hosui A, Miyagi T, Tatsumi T, Ishida H, Ohkawa K, Li W, Kanto T, Hiramatsu N, Hennighausen L, Yin XM, Hayashi N. BH3-only protein bid participates in the Bcl-2 network in healthy liver cells. Hepatology 50: 1972-1980 (2009).

• Hikita H, Takehara T, Shimizu S, Kodama T, Li W, Miyagi T, Hosui A, Ishida H, Ohkawa K, Kanto T, Hiramatsu N, Yin XM, Hennighausen L, Tatsumi T, Hayashi N. Mcl-1 and Bcl-xL cooperatively maintain integrity of hepatocytes in developing and adult murine liver. Hepatology 50: 1217-1226 (2009).

• Hirakata M, Akizuki M, Miyachi K, Matsushima H, Okano T, Homma M. Coexistence of CREST syndrome and primary biliary cirrhosis. Serological studies of two cases. J. Rheumatol. 15: 1166-1170 (1988).

• Hiraoka A, Kojima N, Yamauchi Y, Ninomiya T, Masumoto T, Michitaka K, Horiike N, Onji M. An autopsy case of primary biliary cirrhosis with severe interstitial pneumonia. Intern. Med. 40: 1104-1108 (2001). *

• Hirschfield GM, Heathcote EJ. Antimitochondrial antibody-negative primary biliary cirrhosis. Clin. Liver Dis. 12: 323-331 (2008).

• Hirschfield GM, Liu X, Xu C, Lu Y, Xie G, Lu Y, Gu X, Walker EJ, Jing K, Juran BD, Mason AL, Myers RP, Peltekian KM, Ghent CN, Coltescu C, Atkinson EJ, Heathcote EJ, Lazaridis KN, Amos CI, Siminovitch KA. Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. N. Engl. J. Med. 360: 2544-2555 (2009). *

• Hoffman M, Burke M, Fried M, Turner D, Yosipov Y, Yust I. Primary biliary cirrhosis associated with antiphospholipid syndrome. Isr. J. Med. Sci. 33: 681-686 (1997).

• Hoffmann R, Germann EJ, Schmidt U, Jenss H. Primary biliary liver cirrhosis and granulomatous pulmonary infiltrates. Schweiz. Med. Wochenschr. 116: 1098-1102 (1986).

• Hoffmann U, Heilmann K, Hayford C, Stallmach A, Wahnschaffe U, Zeitz M, Gunthert U, Wittig BM. CD44v7 ligation downregulates the inflammatory immune response in Crohn's disease patients by apoptosis induction in mononuclear cells from the lamina propria. Cell Death Differ. 14: 1542-1551 (2007).

• Hofmanova J, Vaculova A, Hyzd'alova M, Kozubik A. Response of normal and colon cancer epithelial cells to TNF-family apoptotic inducers. Oncol. Rep. 19: 567-573 (2008). *

• Hoglen NC, Hirakawa BP, Fisher CD, Weeks S, Srinivasan A, Wong AM, Valentino KL, Tomaselli KJ, Bai X, Karanewsky DS, Contreras PC. Characterization of the caspase inhibitor IDN-1965 in a model of apoptosis-associated liver injury. J. Pharmacol. Exp. Ther. 297: 811-818 (2001). *

• Hohenester S, Oude-Elferink RP, Beuers U. Primary biliary cirrhosis. Semin. Immunopathol. 31: 283-307 (2009). *

• Hokari A, Zeniya M, Esumi H, Kawabe T, Gershwin ME, Toda G. Detection of serum nitrite and nitrate in primary biliary cirrhosis: possible role of nitric oxide in bile duct injury. J. Gastroenterol. Hepatol. 17: 308-315 (2002). *

• Hollingsworth KG, Newton JL, Taylor R, McDonald C, Palmer JM, Blamire AM, Jones DE. Pilot study of peripheral muscle function in primary biliary cirrhosis: potential implications for fatigue pathogenesis. Clin. Gastroenterol. Hepatol. 6: 1041-1048 (2008).

• Holt KM, Miyai K, Chojkier M. Asymptomatic primary biliary cirrhosis in an old man. J. Clin. Gastroenterol. 8: 203-206 (1986).

• Homberg JC, Abuaf N, Bernard O, Islam S, Alvarez F, Khalil SH, Poupon R, Darnis F, Levy VG, Grippon P, et al. Chronic active hepatitis associated with antiliver/kidney microsome antibody type 1: a second type of "autoimmune" hepatitis. Hepatology 7: 1333-1339 (1987).

• Hong TK, Lee-Kim YC. Effects of retinoic acid isomers on apoptosis and enzymatic antioxidant system in human breast cancer cells. Nutr. Res. Pract. 3: 77-83 (2009). *

• Honma F, Shio K, Monoe K, Kanno Y, Takahashi A, Yokokawa J, Kobayashi H, Watanabe H, Irisawa A, Ohira H. Primary biliary cirrhosis complicated by polymyositis and pulmonary hypertension. Intern. Med. 47: 667-669 (2008). *

• Hopf U, Jahn HU, Moller B, Stemerowicz R, Wittenbrink C, Klein R, Berg PA. Liver membrane antibodies (LMA) recognize a 26-kD protein on the hepatocellular surface. Clin. Exp. Immunol. 79: 54-61 (1990). *

• Hopf U, Moller B, Lobeck H, Klein R, Berg PA. Immunoserologic differentiation of chronic cholestatic hepatitis. Significance of antimitochondrial antibodies and hepatic membrane antibodies. Dtsch. Med. Wochenschr. 110: 1924-1929 (1985).

• Hopf U, Moller B, Stemerowicz R, Lobeck H, Rodloff A, Freudenberg M, Galanos C, Huhn D. Relation between Escherichia coli R(rough)-forms in gut, lipid A in liver, and primary biliary cirrhosis. Lancet 2: 1419-1422 (1989).

• Hopf U, Riecken EO, Zeitz M, Eckhardt R, Lobeck H, Malchus R, Moller B. Immunologic parameters in pericholangitis and primary sclerosing cholangitis with and without ulcerative colitis. Dtsch. Med. Wochenschr. 108: 1515-1521 (1983).

• Hopf U, Stemerowicz R, Knigge O, Khadar R, Konig V, Lobeck H, Neuhaus P. Primary sclerosing cholangitis (PSC) - humoral immune phenomena, pathogenetic aspects and therapeutic possibilities. Z. Gastroenterol. 31 Suppl. 2: 106-110 (1993).

• Hopfner M, Sutter AP, Huether A, Schuppan D, Zeitz M, Scherubl H. Targeting the epidermal growth factor receptor by gefitinib for treatment of hepatocellular carcinoma. J. Hepatol. 41: 1008-1016 (2004). *

• Horigome H, Nomura T, Saso K, Joh T, Ohara H, Akita S, Sobue S, Mizuno Y, Kato Y, Itoh M. Coexistence of primary biliary cirrhosis and myasthenia gravis: a case study. Hepatogastroenterology 47: 125-127 (2000).

• Horsmans Y, Piret A, Brenard R, Rahier J, Geubel AP. Autoimmune chronic active hepatitis responsive to immunosuppressive therapy evolving into a typical primary biliary cirrhosis syndrome: a case report. J. Hepatol. 21: 194-198 (1994).

• Hosoda H, Okawa-Takatsuji M, Tanaka A, Uwatoko S, Aotsuka S, Hasimoto N, Ozaki Y, Ikeda Y. Detection of autoantibody against carbonic anhydrase II in various liver diseases by enzyme-linked immunosorbent assay using appropriate conditions. Clin. Chim. Acta 342: 71-81 (2004). *

• Hosokawa A, Takahashi H, Akaike J, Okuda H, Murakami R, Kawahito Y, Tokuno T, Makiguchi Y, Sakamoto H, Hinoda Y, Imai K. A case of Sjogren's syndrome associated with inflammatory pseudotumor of the liver. Nihon Rinsho Meneki Gakkai Kaishi 21: 226-233 (1998).

• Hosseini-Tabatabaei A, Abdollahi M. Potassium channel openers and improvement of toxic stress: do they have role in the management of inflammatory bowel disease? Inflamm. Allergy Drug Targets 7: 129-135 (2008).

• Hosszufalusi N, Karcagi V, Horvath R, Palik E, Varkonyi J, Rajczy K, Prohaszka Z, Szentirmai C, Karadi I, Romics L, Panczel P. A detailed investigation of maternally inherited diabetes and deafness (MIDD) including clinical characteristics, C-peptide secretion, HLA-DR and -DQ status and autoantibody pattern. Diabetes Metab. Res. Rev. 25: 127-135 (2009). *

• Houston MC. The role of mercury and cadmium heavy metals in vascular disease, hypertension, coronary heart disease, and myocardial infarction. Altern. Ther. Health Med. 13: S128-S133 (2007).

• Howard MJ, Fuller C, Broadhurst RW, Perham RN, Tang JG, Quinn J, Diamond AG, Yeaman SJ. Three-dimensional structure of the major autoantigen in primary biliary cirrhosis. Gastroenterology 115: 139-146 (1998). *

• Hsieh SY, Shih TC, Yeh CY, Lin CJ, Chou YY, Lee YS. Comparative proteomic studies on the pathogenesis of human ulcerative colitis. Proteomics 6: 5322-5331 (2006). *

• Hsu W, Zhang W, Tsuneyama K, Moritoki Y, Ridgway WM, Ansari AA, Coppel RL, Lian ZX, Mackay I, Gershwin ME. Differential mechanisms in the pathogenesis of autoimmune cholangitis versus inflammatory bowel disease in interleukin-2Ralpha(-/-) mice. Hepatology 49: 133-140 (2009). *

• Huang RH, Chai J, Tarnawski AS. Identification of specific genes and pathways involved in NSAIDs-induced apoptosis of human colon cancer cells. World J. Gastroenterol. 12: 6446-6452 (2006). *

• Hug B, Fried R, Terracciano L. Pruritus in liver disease: case report with discussion. Schweiz. Rundsch. Med. Prax. 87: 1805-1810 (1998).

• Hughes DE, Stolz DB, Yu S, Tan Y, Reddy JK, Watkins SC, Diehl AM, Costa RH. Elevated hepatocyte levels of the Forkhead box A2 (HNF-3beta) transcription factor cause postnatal steatosis and mitochondrial damage. Hepatology 37: 1414-1424 (2003). *

• Hulbert AJ, Else PL. Membranes and the setting of energy demand. J. Exp. Biol. 208: 1593-1599 (2005). *

• Hulbert AJ, Faulks SC, Buffenstein R. Oxidation-resistant membrane phospholipids can explain longevity differences among the longest-living rodents and similarly-sized mice. J. Gerontol. A. Biol. Sci. Med. Sci. 61: 1009-1018 (2006).

• Hung HC, Lin CY, Liao YF, Hsu PC, Tsay GJ, Liu GY. The functional haplotype of peptidylarginine deiminase IV (S55G, A82V and A112G) associated with susceptibility to rheumatoid arthritis dominates apoptosis of acute T leukemia Jurkat cells. Apoptosis 12: 475-487 (2007). *

• Hurlburt KJ, McMahon BJ, Deubner H, Hsu-Trawinski B, Williams JL, Kowdley KV. Prevalence of autoimmune liver disease in Alaska Natives. Am. J. Gastroenterol. 97: 2402-2407 (2002). *

• Husby G, Skrede S, Blomhoff JP, Jacobsen CD, Berg K, Gjone E. Serum immunoglobulins and organ non-specific antibodies in diseases of the liver. Scand. J. Gastroenterol. 12: 297-304 (1977).

• Hussaini SH, Farrington EA. Idiosyncratic drug-induced liver injury: an overview. Expert Opin. Drug Saf. 6: 673-684 (2007).

• Hwang SJ, Chan CY, Lee SD, Wu JC, Tsay SH, Lo KJ. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis: a short-term, randomized, double-blind controlled, cross-over study with long-term follow up. J. Gastroenterol. Hepatol. 8: 217-223 (1993).

• Iacobazzi V, Convertini P, Infantino V, Scarcia P, Todisco S, Palmieri F. Statins, fibrates and retinoic acid upregulate mitochondrial acylcarnitine carrier gene expression. Biochem. Biophys. Res. Commun. 388: 643-647 (2009). *

• Ibdah JA, Perlegas P, Zhao Y, Angdisen J, Borgerink H, Shadoan MK, Wagner JD, Matern D, Rinaldo P, Cline JM. Mice heterozygous for a defect in mitochondrial trifunctional protein develop hepatic steatosis and insulin resistance. Gastroenterology 128: 1381-1390 (2005). *

• Ichiki Y, Leung PS, Ishibashi H, Coppel RL, Ansari AA, Gershwin ME. Mitochondria and autoimmunity in primary biliary cirrhosis. Mitochondrion 4: 743-753 (2004).

• Ichiki Y, Selmi C, Shimoda S, Ishibashi H, Gordon SC, Gershwin ME. Mitochondrial antigens as targets of cellular and humoral auto-immunity in primary biliary cirrhosis. Clin. Rev. Allergy Immunol. 28: 83-92 (2005). *

• Ida H, Utz PJ, Anderson P, Eguchi K. Granzyme B and natural killer (NK) cell death. Mod. Rheumatol. 15: 315-322 (2005).

• Ide T, Sata M, Suzuki H, Uchimura Y, Murashima S, Shirachi M, Tanikawa K. An experimental animal model of primary biliary cirrhosis induced by lipopolysaccharide and pyruvate dehydrogenase. Kurume. Med. J. 43: 185-188 (1996).

• Ikeda Y, Toda G, Hashimoto N, Aotsuka S, Yokohari R, Maruyama T, Oka H. Anticalmodulin autoantibody in liver diseases: a new antibody against a cytoskeleton-related protein. Hepatology 7: 285-293 (1987).

• Ikuno N, Mackay IR, Jois J, Omagari K, Rowley MJ. Antimitochondrial autoantibodies in saliva and sera from patients with primary biliary cirrhosis. J. Gastroenterol. Hepatol. 16: 1390-1394 (2001). *

• Il'ichenko LIu, Golovanova EV, Tsaregorodtseva TM, Serova TI, Gudkova RB. Current understanding of primary biliary cirrhosis. Ter. Arkh. 77: 50-54 (2005).

• Ilan Y, Shouval D. Primary biliary cirrhosis in Israel. Isr. J. Med. Sci. 28: 683-687 (1992).

• Imada J, Hoshino H, Nishimura D, Morita K, Yoshida N, Katada N, Sano H, Kato K, Mori N. Case report: multiple cancers: hepatocellular carcinoma and adenocarcinomas of the common bile duct and the gall-bladder in a woman with primary biliary cirrhosis. J. Gastroenterol. Hepatol. 11: 546-550 (1996).

• Imose M, Nagaki M, Naiki T, Osawa Y, Brenner DA, Asano T, Hayashi H, Kato T, Moriwaki H. Inhibition of nuclear factor kappaB and phosphatidylinositol 3-kinase/Akt is essential for massive hepatocyte apoptosis induced by tumor necrosis factor alpha in mice. Liver Int. 23: 386-396 (2003). *

• Inamura K,Tsuji H, Nakamoto Y, Suzuki M, Kaneko S. Transgenic mice aberrantly expressing pyruvate dehydrogenase complex E2 component on biliary epithelial cells do not show primary biliary cirrhosis. Clin. Exp. Immunol. 145: 93-100 (2006). *

• Inbar Y, Zurgil N, Gilburt C, Shoenfeld Y. Antimitochondrial antibodies and primary biliary cirrhosis. Harefuah. 125: 148-152 (1993).

• Ingiliz P, Valantin MA, Duvivier C, Medja F, Dominguez S, Charlotte F, Tubiana R, Poynard T, Katlama C, Lombes A, Benhamou Y. Liver damage underlying unexplained transaminase elevation in human immunodeficiency virus-1 mono-infected patients on antiretroviral therapy. Hepatology 49: 436-442 (2009). *

• Inoue K, Hirohara J, Nakano T, Seki T, Sasaki H, Higuchi K, Ohta Y, Onji M, Muto Y, Moriwaki H. Prediction of prognosis of primary biliary cirrhosis in Japan. Liver 15: 70-77 (1995).

• Inoue T, Shiraki K, Fuke H, Yamanaka Y, Miyashita K, Ito K, Suzuki M, Sugimoto K, Murata K, Nakano T. A case report of primary biliary cirrhosis after aortoiliac reconstruction surgery using a Y-graft. World J. Gastroenterol. 11: 6219-6220 (2005). *

• Invernizzi P. Role of X chromosome defects in primary biliary cirrhosis. Hepatol. Res. 37 Suppl. 3: S384-S388 (2007). *

• Invernizzi P, Battezzati PM, Crosignani A, Perego F, Poli F, Morabito A, De Arias AE, Scalamogna M, Zuin M, Podda M. Peculiar HLA polymorphisms in Italian patients with primary biliary cirrhosis. J. Hepatol. 38: 401-406 (2003). *

• Invernizzi P, Battezzati PM, Crosignani A, Zermiani P, Bignotto M, Del Papa N, Zuin M, Podda M. Antibody to carbonic anhydrase II is present in primary biliary cirrhosis (PBC) irrespective of antimitochondrial antibody status. Clin. Exp. Immunol. 114: 448-454 (1998). *

• Invernizzi P, Crosignani A, Battezzati PM, Covini G, De Valle G, Larghi A, Zuin M, Podda M. Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and -negative primary biliary cirrhosis. Hepatology 25: 1090-1095 (1997). *

• Invernizzi P, Gershwin ME. The genetic basis of primary biliary cirrhosis: premises, not promises. Gastroenterology 135: 1044-1047 (2008). *

• Invernizzi P, Podda M, Battezzati PM, Crosignani A, Zuin M, Hitchman E, Maggioni M, Meroni PL, Penner E, Wesierska-Gadek J. Autoantibodies against nuclear pore complexes are associated with more active and severe liver disease in primary biliary cirrhosis. J. Hepatol. 34: 366-372 (2001). *

• Invernizzi P, Selmi C, Ranftler C, Podda M, Wesierska-Gadek J. Antinuclear antibodies in primary biliary cirrhosis. Semin. Liver Dis. 25: 298-310 (2005).

• Invernizzi P, Selmi C, Zuin M, Podda M. Lack of serum antibodies to membrane bound carbonic anhydrase IV in patients with primary biliary cirrhosis. Gut 54: 1665 (2005). *

• Irestedt M, Svensson A. Genital and extragenital lichen sclerosus et atrophicus in a male patient with hepatosis and anti-mitochondrial antibodies. Br. J. Dermatol. 117: 527-528 (1987).

• Irie J, Wu Y, Wicker LS, Rainbow D, Nalesnik MA, Hirsch R, Peterson LB, Leung PS, Cheng C, Mackay IR, Gershwin ME, Ridgway WM. NOD.c3c4 congenic mice develop autoimmune biliary disease that serologically and pathogenetically models human primary biliary cirrhosis. J. Exp. Med. 203: 1209-1219 (2006). *

• Ishibashi H, Komori A, Shimoda S, Gershwin ME. Guidelines for therapy of autoimmune liver disease. Semin. Liver Dis. 27: 214-226 (2007). *

• Ishibashi H, Shimoda S, Gershwin ME. The immune response to mitochondrial autoantigens. Semin. Liver Dis. 25: 337-346 (2005).

• Ishibashi H, Shimoda S, Nakamura M, Gershwin ME. Chapter 34. Autoimmune diseases in transplanted livers. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 451-457 (2007). *

• Ishigami F, Naka S, Takeshita K, Kurumi Y, Hanasawa K, Tani T. Bile salt tauroursodeoxycholic acid modulation of Bax translocation to mitochondria protects the liver from warm ischemia-reperfusion injury in the rat. Transplantation 72: 1803-1807 (2001).

• Ishii H, Muno D, Namihisa T. Electrophoretic studies of antimitochondrial antibodies: identification of mitochondrial antigens specific to primary biliary cirrhosis. Electrophoresis 10: 612-618 (1989).

• Ishii H, Saifuku K, Namihisa T. Multiplicity of mitochondrial inner membrane antigens from beef heart reacting with antimitochondrial antibodies in sera of patients with primary biliary cirrhosis. Immunol. Lett. 9: 325-330 (1985).

• Ishii H, Saifuku K, Namihisa T. Reactivities and clinical relevance of antimitochondrial antibodies to four mitochondrial inner membrane proteins in sera of patients with primary biliary cirrhosis. Hepatology 7: 134-136 (1987).

• Ishikawa T, Kakumu S, Wakita T, Takayanagi M, Yoshioka K. In vitro production of antimitochondrial antibody by peripheral blood mononuclear cells in patients with primary biliary cirrhosis. J. Clin. Lab. Immunol. 35: 17-25 (1991).

• Ito Y, Yamanoshita O, Kurata Y, Kamijima M, Aoyama T, Nakajima T. Induction of peroxisome proliferator-activated receptor alpha (PPARalpha)-related enzymes by di(2-ethylhexyl) phthalate (DEHP) treatment in mice and rats, but not marmosets. Arch. Toxicol. 81: 219-226 (2007). *

• Itoh S, Ichida T, Yoshida T, Hayakawa A, Uchida M, Tashiro-Itoh T, Matsuda Y, Ishihara K, Asakura H. Autoantibodies against a 210 kDa glycoprotein of the nuclear pore complex as a prognostic marker in patients with primary biliary cirrhosis. J. Gastroenterol. Hepatol. 13: 257-265 (1998).

• Itoh S, Matsuzaki Y, Kimura T, Ikegami T, Shoda J, Fujiwara M, Tanaka N. Cytokine profile of liver-infiltrating CD4+ T cells separated from murine primary biliary cirrhosis-like hepatic lesions induced by graft-versus-host reaction. J. Gastroenterol. Hepatol. 15: 443-451 (2000). *

• Iwasaki S, Tsuda K, Ueta H, Aono R, Ono M, Saibara T, Maeda T, Onishi S. Bezafibrate may have a beneficial effect in pre-cirrhotic primary biliary cirrhosis. Hepatol. Res. 16: 12-18 (1999).

• Iwayama T, Leung PS, Rowley M, Munoz S, Nishioka M, Nakagawa T, Dickson ER, Coppel RL, Mackay IR, Gershwin ME. Comparative immunoreactive profiles of Japanese and American patients with primary biliary cirrhosis against mitochondrial autoantigens. Int. Arch. Allergy Immunol. 99: 28-33 (1992).

• Izumi Y, Tanaka S, Hidaka Y, Shimaoka Y, Tatsumi KI, Takano T, Kaneko A, Oku K, Amino N. Relation between post-partum liver dysfunction and anti-cytochrome 2D6 antibodies. Am. J. Reprod. Immunol. 50: 355-362 (2003). *

• James LP, Mayeux PR, Hinson JA. Acetaminophen-induced hepatotoxicity. Drug Metab. Dispos. 31: 1499-1506 (2003). *

• James O, Macklon AF, Watson AJ. Primary biliary cirrhosis - a revised clinical spectrum. Lancet 1: 1278-1281 (1981).

• Jang JY, Choi Y, Jeon YK, Kim CW. The suppression of adenine nucleotide translocase-2 (ANT2) by vector-based siRNA in human breast cancer cells induces apoptosis and inhibits tumor growth in vitro and in vivo. Breast Cancer Res. 10: R11 (2008). *

• Jazia EB, Khalifa M, Abdelkader AB, Kaabia N, Ghannouchi N, Braham A, Letaief A, Bahri F. A case of primary biliary cirrhosis associated with pernicious anemia: a case report. Cases J. 3: 11 (2010). *

• Jefcoate CR. Liver X receptor opens a new gateway to StAR and to steroid hormones. J. Clin. Invest. 116: 1832-1835 (2006). *

• Jensen WA, Jois JA, Murphy P, De Giorgio J, Brown B, Rowley MJ, Mackay IR. Automated enzymatic mitochondrial antibody assay for the diagnosis of primary biliary cirrhosis. Clin. Chem. Lab. Med. 38: 753-758 (2000).

• Jia Y, Song T, Wei C, Ni C, Zheng Z, Xu Q, Ma H, Li L, Zhang Y, He X, Xu Y, Shi W, Zhong H. Negative regulation of MAVS-mediated innate immune response by PSMA7. J. Immunol. 183: 4241-4248 (2009).

• Jiang H, Chen K, He J, Pan F, Li J, Chen J, Chen W, Liang H. Association of pregnane X receptor with multidrug resistance-related protein 3 and its role in human colon cancer chemoresistance. J. Gastrointest. Surg. 13: 1831-1838 (2009). *

• Jiang T, Han Z, Chen S, Wu C, Tang Y, Qian C, Chen Y, Zhou Y, Zhu Y, Gu M, Zhu L, Yao D, Deng A, Zhong R. Resistance to activation-induced cell death and elevated FLIP(L) expression of CD4+ T cells in a polyI:C-induced primary biliary cirrhosis mouse model. Clin. Exp. Med. 9: 269-276 (2009). *

• Jiang XH, Zhong RQ, Yu SQ, Hu Y, Li WW, Kong XT. Construction and expression of a humanized M2 autoantigen trimer and its application in the diagnosis of primary biliary cirrhosis. World J. Gastroenterol. 9: 1352-1355 (2003). *

• Jin X, Zimmers TA, Perez EA, Pierce RH, Zhang Z, Koniaris LG. Paradoxical effects of short- and long-term interleukin-6 exposure on liver injury and repair. Hepatology 43: 474-484 (2006). *

• Johannesen J. Aspects of the molecular and functional genetics in T1DM - a study of selected candidate genes. Dan. Med. Bull. 53: 122-171 (2006).

• Johnston DE, Kaplan MM, Miller KB, Connors CM, Milford EL. Histocompatibility antigens in primary biliary cirrhosis. Am. J. Gastroenterol. 82: 1127-1129 (1987).

• Jois J, Omagari K, Rowley MJ, Anderson J, Mackay IR. Enzyme inhibitory antibody to pyruvate dehydrogenase: diagnostic utility in primary biliary cirrhosis. Ann. Clin. Biochem. 37: 67-73 (2000).

• Jones DE. Autoantigens in primary biliary cirrhosis. J. Clin. Pathol. 53: 813-821 (2000). *

• Jones DE, James OF, Bassendine MF. Primary biliary cirrhosis: clinical and associated autoimmune features and natural history. Clin. Liver Dis. 2: 265-282, viii (1998).

• Jones DE, Palmer JM, Kirby JA, De Cruz DJ, McCaughan GW, Sedgwick JD, Yeaman SJ, Burt AD, Bassendine MF. Experimental autoimmune cholangitis: a mouse model of immune-mediated cholangiopathy. Liver 20: 351-356 (2000).

• Jong-Hon K, Yajima R, Karino Y, Yoshida J, Tsuji K, Watanabe S, Horada K, Itoh Y, Sekiguchi K, Toyoda S, Maekubo H. Development of a new enzyme-linked immunosorbent assay for the detection of anti-M2 in primary biliary cirrhosis. Hepatol. Res. 21: 1-7 (2001).

• Joo Y, Kim HS, Woo RS, Park CH, Shin KY, Lee JP, Chang KA, Kim S, Suh YH. Mefenamic acid shows neuroprotective effects and improves cognitive impairment in in vitro and in vivo Alzheimer's disease models. Mol. Pharmacol. 69: 76-84 (2006). *

• Joplin R. Infectious etiology of primary biliary cirrhosis? Falk Symposium 136. XII FALK LIVER WEEK 2003 (Part I) In Honour of Hans Popper’s 100th Birthday. Cholestatic Liver Diseases: Therapeutical Options and Perspectives. Freiburg (Germany). p. 19-20. October 15-16 (2003). *

• Joplin R, Gershwin ME. Ductular expression of autoantigens in primary biliary cirrhosis. Semin. Liver Dis. 17: 97-103 (1997).

• Joplin R, Lindsay JG, Hubscher SG, Johnson GD, Shaw JC, Strain AJ, Neuberger JM. Distribution of dihydrolipoamide acetyltransferase (E2) in the liver and portal lymph nodes of patients with primary biliary cirrhosis: an immunohistochemical study. Hepatology 14: 442-447 (1991).

• Joplin R, Lindsay JG, Johnson GD, Strain A, Neuberger J. Membrane dihydrolipoamide acetyltransferase (E2) on human biliary epithelial cells in primary biliary cirrhosis. Lancet 339: 93-94 (1992).

• Joplin R, Wallace LL, Johnson GD, Lindsay JG, Yeaman SJ, Palmer JM, Strain AJ, Neuberger JM. Subcellular localization of pyruvate dehydrogenase dihydrolipoamide acetyltransferase in human intrahepatic biliary epithelial cells. J. Pathol. 176: 381-390 (1995).

• Joplin RE, Johnson GD, Matthews JB, Hamburger J, Lindsay JG, Hubscher SG, Strain AJ, Neuberger JM. Distribution of pyruvate dehydrogenase dihydrolipoamide acetyltransferase (PDC-E2) and another mitochondrial marker in salivary gland and biliary epithelium from patients with primary biliary cirrhosis. Hepatology 19: 1375-1380 (1994).

• Joplin RE, Neuberger JM. Immunopathology of primary biliary cirrhosis. Eur. J. Gastroenterol. Hepatol. 11: 587-593 (1999).

• Joplin RE, Wallace LL, Lindsay JG, Palmer JM, Yeaman SJ, Neuberger JM. The human biliary epithelial cell plasma membrane antigen in primary biliary cirrhosis: pyruvate dehydrogenase X? Gastroenterology 113: 1727-1733 (1997). *

• Jorde R, Rekvig OP, Bostad L. A follow-up study of 68 patients with anti-mitochondrial antibodies (AMA). Acta Med. Scand. 220: 241-247 (1986).

• Joshi S, Cauch-Dudek K, Heathcote EJ, Lindor K, Jorgensen R, Klein R. Antimitochondrial antibody profiles: are they valid prognostic indicators in primary biliary cirrhosis? Am. J. Gastroenterol. 97: 999-1002 (2002). *

• Joshi S, Cauch-Dudek K, Wanless IR, Lindor KD, Jorgensen R, Batts K, Heathcote EJ. Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid. Hepatology 35: 409-413 (2002). *

• Juel VC, Massey JM. Myasthenia gravis. Orphanet J. Rare Dis. 2: 44 (2007). *

• Julia E, Edo MC, Horga A, Montalban X, Comabella M. Differential susceptibility to apoptosis of CD4+T cells expressing CCR5 and CXCR3 in patients with MS. Clin. Immunol. 133: 364-374 (2009). *

• Jump DB. Dietary polyunsaturated fatty acid regulation of hepatic gene transcription. Scand. J. Nutr. 46: 59-67 (2002). *

• Juran BD, Atkinson EJ, Larson JJ, Schlicht EM, Lazaridis KN. Common genetic variation and haplotypes of the anion exchanger SLC4A2 in primary biliary cirrhosis. Am. J. Gastroenterol. 104: 1406-1411 (2009). *

• Juran BD, Atkinson EJ, Schlicht EM, Fridley BL, Lazaridis KN. Primary biliary cirrhosis is associated with a genetic variant in the 3' flanking region of the CTLA4 gene. Gastroenterology 135: 1200-1206 (2008). *

• Juran BD, Atkinson EJ, Schlicht EM, Fridley BL, Petersen GM, Lazaridis KN. Interacting alleles of the coinhibitory immunoreceptor genes cytotoxic T-lymphocyte antigen 4 and programmed cell-death 1 influence risk and features of primary biliary cirrhosis. Hepatology 47: 563-570 (2008). *

• Kadaja L, Kisand KE, Peet N, Braun U, Metskua K, Teesalu K, Vibo R, Kisand KV, Uibo R, Jockusch H, Seppet EK. IgG from patients with liver diseases inhibit mitochondrial respiration in permeabilized oxidative muscle cells: impaired function of intracellular energetic units? Mol. Cell. Biochem. 256: 291-303 (2004). *

• Kadokawa Y, Omagari K, Hazama H, Ohba K, Masuda J, Kinoshita H, Hayashida K, Isomoto H, Mizuta Y, Murase K, Murata I, Kohno S. Evaluation of newly developed ELISA using "MESACUP-2 test mitochondrial M2" kit for the diagnosis of primary biliary cirrhosis. Clin. Biochem. 36: 203-210 (2003). *

• Kadokawa Y, Omagari K, Ohba K, Masuda J, Hazama H, Kinoshita H, Ohnita K, Mizuta Y, Tanioka H, Imanishi T, Kohno S. Does the diagnosis of primary biliary cirrhosis or autoimmune cholangitis depend on the 'phase' of the disease? Liver Int. 25: 317-324 (2005). *

• Kadotani A, Tsuchiya Y, Hatakeyama H, Katagiri H, Kanzaki M. Different impacts of saturated and unsaturated free fatty acids on COX-2 expression in C2C12 myotubes. Am. J. Physiol. Endocrinol. Metab. 297: E1291-E1303 (2009). *

• Kai S, Yakushiji K, Yamauchi M, Ito C, Kuwajima M, Osada Y, Toshimori K. Expression of novel organic cation/carnitine transporter (OCTN2) in the mouse pancreas. Tissue Cell. 37: 309-315 (2005). *

• Kakar S, Batts KP, Poterucha JJ, Burgart LJ. Histologic changes mimicking biliary disease in liver biopsies with venous outflow impairment. Mod. Pathol. 17: 874-878 (2004). *

• Kamdar O, Le W, Zhang J, Ghio AJ, Rosen GD, Upadhyay D. Air pollution induces enhanced mitochondrial oxidative stress in cystic fibrosis airway epithelium. FEBS Lett. 582: 3601-3606 (2008). *

• Kamimura T, Yoshida T, Ito S, Tashiro T. Primary biliary cirrhosis: symptoms, laboratory data and diagnosis. Nippon Rinsho 52: 246-250 (1994).

• Kananathan R, Suresh RL, Merican I. Primary biliary cirrhosis at Hospital Kuala Lumpur: a study of 17 cases seen between 1992 and 1999. Med. J. Malaysia 57: 56-60 (2002).

• Kaplan MM. Novosphingobium aromaticivorans: a potential initiator of primary biliary cirrhosis. Am. J. Gastroenterol. 99: 2147-2149 (2004). *

• Kaplan MM, Bianchi DW. Primary biliary cirrhosis: for want of an X chromosome? Lancet 363: 505-506 (2004). *

• Kaplan MM, Gandolfo JV, Quaroni EG. An enzyme-linked immunosorbent assay (ELISA) for detecting antimitochondrial antibody. Hepatology 4: 727-730 (1984).

• Kaplan MM, Knox TA. Treatment of primary biliary cirrhosis with low-dose weekly methotrexate. Gastroenterology 101: 1332-1338 (1991).

• Karlsson-Parra A, Totterman TH, Nyberg A, Mendel-Hartvig I, Loof L, Forsum U. Immunological effects of ciclosporin in primary biliary cirrhosis: suppression of activated T cells and autoantibody levels. Int. Arch. Allergy Appl. Immunol. 83: 256-264 (1987).

• Kaserer K, Exner M, Mosberger I, Penner E, Wrba F. Characterization of the inflammatory infiltrate in autoimmune cholangitis. A morphological and immunhistochemical study. Virchows Arch. 432: 217-222 (1998). *

• Kato T, Miyakawa H, Ishibashi M. Frequency and significance of anti-glutathione S-transferase autoantibody (anti-GST A1-1) in autoimmune hepatitis. J. Autoimmun. 22: 211-216 (2004).

• Kato Y, Suzuki K, Kumagai M, Nishimura K, Miyamori H, Kobayashi K, Hattori N, Nakanuma Y. Familial primary biliary cirrhosis. Immunological and genetic study. Am. J. Gastroenterol. 75: 188-191 (1981).

• Katono S. A comparative study of passive hemagglutination and immunofluorescence methods for the detection of mitochondrial antibodies in sera from patients with liver diseases. Gastroenterol. Jpn. 15: 233-240 (1980).

• Kawaguchi N, Miyakawa H, Abe K, Kitazawa E, Fujikawa H, Kikuchi K, Kako M, Shibata M, Shibuya A, Onozuka Y, Yoshida N, Ueno Y. Western blot analysis of anti-M2 antibodies in anti-mitochondrial antibody-negative primary biliary cirrhosis. Rinsho Byori 45: 1079-1084 (1997).

• Kawaguchi N, Miyakawa H, Abe K, Kitazawa E, Kako M. Establishment of the optimal conditions for detecting anti-M2 by western blotting in primary biliary cirrhosis. Rinsho Byori 45: 1072-1078 (1997).

• Kawai T, Hosoya N, Moritoki Y, Kajiyama Y, Watanabe M, Takai A, Selmi C, Gershwin ME, Miyakawa H, Kikuchi K. Autoantibody IgG subclasses to recombinant antigens and the role of bacterial stimuli in primary biliary cirrhosis. Hepatol. Res. 39: 874-881 (2009). *

• Kaya S, Demirci M, Sesli Cetin E, Cicioglu Aridogan B, Sahin M, Tas T, Korkmaz M. Investigation of the presence of autoantibodies in patients with toxocariasis. Mikrobiyol. Bul. 43: 661-666 (2009).

• Kazama H, Ricci JE, Herndon JM, Hoppe G, Green DR, Ferguson TA. Induction of immunological tolerance by apoptotic cells requires caspase-dependent oxidation of high-mobility group box-1 protein. Immunity 29: 21-32 (2008). *

• Kazuta Y, Watanabe N, Sagawa K, Kobayashi H, Kojima T, Funabashi H, Moritoh T, Kasukawa R. A case of autoimmune hemolytic anemia induced by IFN-beta therapy for type-C chronic hepatitis. Fukushima J. Med. Sci. 41: 43-49 (1995).

• Keeffe EB, Davis GL. Advances in liver disease: highlights from the 54th Annual Meeting of the American Association for the Study of Liver Diseases, October 24-28, 2003. Rev. Gastroenterol. Disord. 4: 36-42 (2004). *

• Kemmis CM, Welsh J. Mammary epithelial cell transformation is associated with deregulation of the vitamin D pathway. J. Cell. Biochem. 105: 980-988 (2008). *

• Kenna JG, Neuberger J, Davies E, Eddleston AL, Williams R. A simple enzyme-linked immunosorbent assay for detection of anti-mitochondrial antibodies. J. Immunol. Methods 73: 401-413 (1984).

• Kenny RP, Czaja AJ, Ludwig J, Dickson ER. Frequency and significance of antimitochondrial antibodies in severe chronic active hepatitis. Dig. Dis. Sci. 31: 705-711 (1986).

• Khoo UY, Bjarnason I, Donaghy A, Williams R, Macpherson A. Antibodies to colonic epithelial cells from the serum and colonic mucosal washings in ulcerative colitis. Gut 37: 63-70 (1995). *

• Kikuchi K, Hsu W, Hosoya N, Moritoki Y, Kajiyama Y, Kawai T, Takai A, Hayam E, Selmi C, Gershwin ME, Miyakawa H. Ursodeoxycholic acid reduces CpG-induced IgM production in patients with primary biliary cirrhosis. Hepatol. Res. 39: 448-454 (2009). *

• Kikuchi K, Lian ZX, Kimura Y, Selmi C, Yang GX, Gordon SC, Invernizzi P, Podda M, Coppel RL, Ansari AA, Ikehara S, Miyakawa H, Gershwin ME. Genetic polymorphisms of Toll-like receptor 9 influence the immune response to CpG and contribute to hyper-IgM in primary biliary cirrhosis. J. Autoimmun. 24: 347-352 (2005).

• Kikuchi K, Lian ZX, Yang GX, Ansari AA, Ikehara S, Kaplan M, Miyakawa H, Coppel RL, Gershwin ME. Bacterial CpG induces hyper-IgM production in CD27+ memory B cells in primary biliary cirrhosis. Gastroenterology 128: 304-312 (2005). *

• Kim HJ, Park KG, Yoo EK, Kim YH, Kim YN, Kim HS, Kim HT, Park JY, Lee KU, Jang WG, Kim JG, Kim BW, Lee IK. Effects of PGC-1alpha on TNF-alpha-induced MCP-1 and VCAM-1 expression and NF-kappaB activation in human aortic smooth muscle and endothelial cells. Antioxid. Redox Signal. 9: 301-307 (2007).

• Kim JH, Kim BH, Kim YW, Park JC, Jung YH, Lee BO, Han YS, Dong SH, Kim HJ, Chang YW, Lee JI, Chang R. Autoimmune cholangitis in a patient with thymoma. J. Gastroenterol. Hepatol. 19: 1324-1327 (2004). *

• Kim WR, Ludwig J, Lindor KD. Variant forms of cholestatic diseases involving small bile ducts in adults. Am. J. Gastroenterol. 95: 1130-1138 (2000). *

• Kim WR, Poterucha JJ, Jorgensen RA, Batts KP, Homburger HA, Dickson ER, Krom RA, Wiesner RH, Lindor KD. Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation. Hepatology 26: 22-26 (1997). *

• Kimura T, Suzuki K, Inada S, Hayashi A, Isobe M, Matsuzaki Y, Tanaka N, Osuga T, Fujiwara M. Monoclonal antibody against lymphocyte function-associated antigen 1 inhibits the formation of primary biliary cirrhosis-like lesions induced by murine graft-versus-host reaction. Hepatology 24: 888-894 (1996). *

• Kimura T, Suzuki K, Inada S, Hayashi A, Saito H, Miyai T, Ohsugi Y, Matsuzaki Y, Tanaka N, Osuga T, et al. Induction of autoimmune disease by graft-versus-host reaction across MHC class II difference: modification of the lesions in IL-6 transgenic mice. Clin. Exp. Immunol. 95: 525-529 (1994). *

• Kingham JG, Parker DR. The association between primary biliary cirrhosis and coeliac disease: a study of relative prevalences. Gut 42: 120-122 (1998). *

• Kinoshita H, Omagari K, Whittingham S, Kato Y, Ishibashi H, Sugi K, Yano M, Kohno S, Nakanuma Y, Penner E, Wesierska-Gadek J, Reynoso-Paz S, Gershwin ME, Anderson J, Jois JA, Mackay IR. Autoimmune cholangitis and primary biliary cirrhosis - an autoimmune enigma. Liver 19: 122-128 (1999).

• Kiriyama K, Usui T, Takemura T, Ikeda K, Kishida H, Izumoto T, Kanayama Y, Hirota S. A case of rapid-onset antimitochondrial antibody-positive autoimmune hepatitis possibly triggered by a urinary tract infection. Nippon Shokakibyo Gakkai Zasshi 107: 270-277 (2010).

• Kisand K, Kisand K, Salupere V, Uibo R. Enzyme-linked immunosorbent assays for the determination of IgG, IgA, and IgM autoantibodies to pyruvate dehydrogenase in primary biliary cirrhosis. Int. J. Clin. Lab. Res. 24: 98-101 (1994).

• Kisand KE, Kisand KV, Karvonen AL, Vuoristo M, Mattila J, Makinen J, Uibo R. Antibodies to pyruvate dehydrogenase in primary biliary cirrhosis: correlation with histology. APMIS 106: 884-892 (1998).

• Kisand KE, Metskula K, Kisand KV, Kivik T, Gershwin ME, Uibo R. The follow-up of asymptomatic persons with antibodies to pyruvate dehydrogenase in adult population samples. J. Gastroenterol. 36: 248-254 (2001). *

• Kita H, Ansari AA, He XS, Lian ZX, Van de Water J, Coppel RL, Luketic V, Kaplan M, Inamori H, Isoda N, Sugano K, Imawari M, Gershwin ME. Proteasome is required for class I-restricted presentation by Fcgamma receptor-mediated endocytosis in primary biliary cirrhosis. J. Autoimmun. 21: 175-182 (2003). *

• Kita H, Lian ZX, Van de Water J, He XS, Matsumura S, Kaplan M, Luketic V, Coppel RL, Ansari AA, Gershwin ME. Identification of HLA-A2-restricted CD8(+) cytotoxic T cell responses in primary biliary cirrhosis: T cell activation is augmented by immune complexes cross-presented by dendritic cells. J. Exp. Med. 195: 113-123 (2002). *

• Kita H, Mackay IR, Van De Water J, Gershwin ME. The lymphoid liver: considerations on pathways to autoimmune injury. Gastroenterology 120: 1485-1501 (2001). *

• Kita H, Nalbandian G, Keeffe EB, Coppel RL, Gershwin ME. Pathogenesis of primary biliary cirrhosis. Clin. Liver Dis. 7: 821-839 (2003).

• Kitami N, Ishii H, Shimizu H, Adachi H, Komada T, Mikami H, Yokoi Y, Sato N. Immunoreactivity to M2 proteins in antimitochondrial antibody-negative patients with primary biliary cirrhosis. J. Gastroenterol. Hepatol. 9: 7-12 (1994).

• Kitami N, Komada T, Ishii H, Shimizu H, Adachi H, Yamaguchi Y, Kitamura T, Oide H, Miyazaki A, Ishikawa M, et al. Immunological study of anti-M2 in antimitochondrial antibody-negative primary biliary cirrhosis. Intern. Med. 34: 496-501 (1995). *

• Klassen LW, Thiele GM. Chapter 24. The immune response in the pathogenesis of alcoholic liver disease. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 309-321 (2007). *

• Klein R, Berg PA. Prognostic significance of antimitochondrial antibody profiles A-D in primary biliary cirrhosis. Dtsch. Med. Wochenschr. 113: 1549-1553 (1988).

• Klein R, Berg PA. Anti-M9 antibodies in sera from patients with primary biliary cirrhosis recognize an epitope of glycogen phosphorylase. Clin. Exp. Immunol. 81: 65-71 (1990). *

• Klein R, Berg PA. Anti-M4 antibodies in primary biliary cirrhosis react with sulphite oxidase, an enzyme of the mitochondrial inter-membrane space. Clin. Exp. Immunol. 84: 445-448 (1991). *

• Klein R, Berg PA. Demonstration of "naturally occurring mitochondrial antibodies" in family members of patients with primary biliary cirrhosis. Hepatology 12: 335-341 (1990).

• Klein R, Berg PA. Characterization of a new mitochondrial antigen-antibody system (M9/anti-M9) in patients with anti-M2 positive and anti-M2 negative primary biliary cirrhosis. Clin. Exp. Immunol. 74: 68-74 (1988). *

• Klein R, Huizenga JR, Gips CH, Berg PA. Antimitochondrial antibody profiles in patients with primary biliary cirrhosis before orthotopic liver transplantation and titres of antimitochondrial antibody-subtypes after transplantation. J. Hepatol. 20: 181-189 (1994).

• Klein R, Kloppel G, Fischer R, Fintelmann V, Muting D, Berg PA. The antimitochondrial antibody anti-M9. A marker for the diagnosis of early primary biliary cirrhosis. J. Hepatol. 6: 299-306 (1988).

• Klein R, Kloppel G, Garbe W, Fintelmann V, Berg PA. Antimitochondrial antibody profiles determined at early stages of primary biliary cirrhosis differentiate between a benign and a progressive course of the disease. A retrospective analysis of 76 patients over 6-18 years. J. Hepatol. 12: 21-27 (1991).

• Klein R, Lindenborn-Fotinos J, Berg PA. Use of ATPase-associated antigen (M2) for detection of antimitochondrial antibodies in primary biliary cirrhosis by fluorometric immunoassay. J. Immunol. Methods 64: 227-238 (1983).

• Klein R, Pointner H, Zilly W, Glassner-Bittner B, Breuer N, Garbe W, Fintelmann V, Kalk JF, Muting D, Fischer R, Tittor W, Pausch J, Maier KP, Berg PA. Antimitochondrial antibody profiles in primary biliary cirrhosis distinguish at early stages between a benign and a progressive course: a prospective study on 200 patients followed for 10 years. Liver 17: 119-128 (1997).

• Klein R, Wiebel M, Engelhart S, Berg PA. Sera from patients with tuberculosis recognize the M2a-epitope (E2-subunit of pyruvate dehydrogenase) specific for primary biliary cirrhosis. Clin. Exp. Immunol. 92: 308-316 (1993). *

• Kloppel G, Kirchhof M, Berg PA. Natural course of primary biliary cirrhosis. I. A morphological, clinical and serological analysis of 103 cases. Liver 2: 141-151 (1982).

• Kloppel G, Seifert G, Lindner H, Dammermann R, Sack HJ, Berg PA. Histopathological features in mixed types of chronic aggressive hepatitis and primary biliary cirrhosis. Correlations of liver histology with mitochondrial antibodies of different specificity. Virchows Arch. A. Pathol. Anat. Histol. 373: 143-160 (1977).

• Ko GT, Szeto CC, Yeung VT, Chow CC, Chan H, Cockram CS. Autoimmune polyglandular syndrome and primary biliary cirrhosis. Br. J. Clin. Pract. 50: 344-346 (1996).

• Kobashi H, Yamamoto K, Yoshioka T, Tomita M, Tsuji T. Nonsuppurative cholangitis is induced in neonatally thymectomized mice: a possible animal model for primary biliary cirrhosis. Hepatology 19: 1424-1430 (1994).

• Kodama T, Imai H, Wakui H, Ohtani H, Komatsuda A, Miura AB. Tubulointerstitial nephritis with renal tubular acidosis and asymptomatic primary biliary cirrhosis accompanied by antibody to a 52-kDa mitochondrial protein alone. Clin. Nephrol. 45: 401-405 (1996).

• Kogawa H, Migita K, Ito M, Takii Y, Daikoku M, Nakao M, Miyashita T, Kimura H, Ezaki H, Nakamura M, Yatsuhashi H, Eguchi K, Ishibashi H. Idiopathic portal hypertension associated with systemic sclerosis and Sjogren's syndrome. Clin. Rheumatol. 24: 544-547 (2005). *

• Koh WH, Dunphy J, Whyte J, Dixey J, McHugh NJ. Characterisation of anticytoplasmic antibodies and their clinical associations. Ann. Rheum. Dis. 54: 269-273 (1995). *

• Koh YJ, Park BH, Park JH, Han J, Lee IK, Park JW, Koh GY. Activation of PPARgamma induces profound multilocularization of adipocytes in adult mouse white adipose tissues. Exp. Mol. Med. Sep 11 [Epub ahead of print] (2009). *

• Kohjima M, Enjoji M, Higuchi N, Kato M, Kotoh K, Yoshimoto T, Fujino T, Yada M, Yada R, Harada N, Takayanagi R, Nakamuta M. Re-evaluation of fatty acid metabolism-related gene expression in nonalcoholic fatty liver disease. Int. J. Mol. Med. 20: 351-358 (2007). *

• Kohli M, Yu J, Seaman C, Bardelli A, Kinzler KW, Vogelstein B, Lengauer C, Zhang L. SMAC/Diablo-dependent apoptosis induced by nonsteroidal antiinflammatory drugs (NSAIDs) in colon cancer cells. Proc. Natl. Acad. Sci. U.S.A. 101: 16897-16902 (2004). *

• Koike K, Ishibashi H, Koike M. Immunoreactivity of porcine heart dihydrolipoamide acetyl- and succinyl-transferases (PDC-E2, OGDC-E2) with primary biliary cirrhosis sera: characterization of the autoantigenic region and effects of enzymatic delipoylation and relipoylation. Hepatology 27: 1467-1474 (1998). *

• Kolar SS, Barhoumi R, Callaway ES, Fan YY, Wang N, Lupton JR, Chapkin RS. Synergy between docosahexaenoic acid and butyrate elicits p53-independent apoptosis via mitochondrial Ca2+ accumulation in colonocytes. Am. J. Physiol. Gastrointest. Liver Physiol. 293: G935-G943 (2007). *

• Komatsu T, Utsunomiya K, Oyaizu T. Goodpasture's syndrome associated with primary biliary cirrhosis. Intern. Med. 37: 611-613 (1998). *

• Komita H, Homma S, Saotome H, Zeniya M, Ohno T, Toda G. Interferon-gamma produced by interleukin-12-activated tumor infiltrating CD8+T cells directly induces apoptosis of mouse hepatocellular carcinoma. J. Hepatol. 45: 662-672 (2006). *

• Kong X, Fan H, Liu X, Wang R, Liang J, Gupta N, Chen Y, Fang F, Chang Y. Peroxisome proliferator-activated receptor gamma coactivator-1 alpha enhances anti-proliferative activity of 5'-deoxy-5-fluorouridine in cancer cells through induction of uridine phosphorylase. Mol. Pharmacol. 76: 854-860 (2009). *

• Korkut E, Kisacik B, Akcan Y, Belenli O, Bicik Z, Yucel OG. Two successive pregnancies after ursodeoxycholic acid therapy in a previously infertile woman with antimitochondrial antibody-negative primary biliary cirrhosis. Fertil. Steril. 83: 761-763 (2005). *

• Koulentaki M, Moscandrea J, Dimoulios P, Chatzicostas C, Kouroumalis EA. Survival of anti-mitochondrial antibody-positive and -negative primary biliary cirrhosis patients on ursodeoxycholic acid treatment. Dig. Dis. Sci. 49: 1190-1195 (2004). *

• Kouroumalis E, Notas G. Pathogenesis of primary biliary cirrhosis: a unifying model. World J. Gastroenterol. 12: 2320-2327 (2006). *

• Koutsoumpas A, Mytilinaiou M, Polymeros D, Dalekos GN, Bogdanos DP. Anti-Helicobacter pylori antibody responses specific for VacA do not trigger primary biliary cirrhosis-specific antimitochondrial antibodies. Eur. J. Gastroenterol. Hepatol. 21: 1220 (2009).

• Krammer PH. Apoptosis in the immune system. Falk Symposium 142: GASTROENTEROLOGY WEEK FREIBURG 2004 (Part I) AUTOIMMUNE LIVER DISEASE, Freiburg (Germany), October 12 - 13, 2004, p. 25 (2004). *

• Krams SM, Cao S, Hayashi M, Villanueva JC, Martinez OM. Elevations in IFN-gamma, IL-5, and IL-10 in patients with the autoimmune disease primary biliary cirrhosis: association with autoantibodies and soluble CD30. Clin. Immunol. Immunopathol. 80: 311-320 (1996). *

• Krams SM, Dorshkind K, Gershwin ME. Generation of biliary lesions after transfer of human lymphocytes into severe combined immunodeficient (SCID) mice. J. Exp. Med. 170: 1919-1930 (1989). *

• Krams SM, Surh CD, Coppel RL, Ansari A, Ruebner B, Gershwin ME. Immunization of experimental animals with dihydrolipoamide acetyltransferase, as a purified recombinant polypeptide, generates mitochondrial antibodies but not primary biliary cirrhosis. Hepatology 9: 411-416 (1989).

• Kratzsch KH, Zimmermann S, Morenz J, Furstenau M, Zugehor M, Claus B, Lewicki I. Humoral immune reaction in chronic liver diseases. Z. Gesamte. Inn. Med. 31: 533-545 (1976).

• Kremer AE, Rust C, Eichhorn P, Beuers U, Holdenrieder S. Immune-mediated liver diseases: programmed cell death ligands and circulating apoptotic markers. Expert Rev. Mol. Diagn. 9: 139-156 (2009).

• Kretschmer NW, Boor PJ, el Azhary RA, Ahmed AE, Reynolds ES. Studies on the mechanism of 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU)-induced hepatotoxicity. III. Ultrastructural characterization of bile duct injury. Cancer Chemother. Pharmacol. 19: 109-117 (1987).

• Krulik M, Zylberait D, Vittecoq D, Audebert AA, Debray J. Primary biliary cirrhosis associated with acute disseminated lupus erythematosus. Nouv. Presse. Med. 9: 31-34 (1980).

• Kubota J, Ikeda F, Terada R, Kobashi H, Fujioka SI, Okamoto R, Baba S, Morimoto Y, Ando M, Makino Y, Taniguchi H, Yasunaka T, Miyake Y, Iwasaki Y, Yamamoto K. Mortality rate of patients with asymptomatic primary biliary cirrhosis diagnosed at age 55 years or older is similar to that of the general population. J. Gastroenterol. 44: 1000-1006 (2009). *

• Kuiper EM, den Ouden-Muller JW, van Buuren HR. Primary biliary cirrhosis. Ned. Tijdschr. Geneeskd. 153: A483 (2009).

• Kuloglu Z, Kirsaclioglu CT, Kansu A, Ensari A, Girgin N. Celiac disease: presentation of 109 children. Yonsei Med. J. 50: 617-623 (2009). *

• Kumagi T, Heathcote JE. Primary biliary cirrhosis. Orphanet J. Rare Dis. 3: 1 (2008). *

• Kurdow R, Marks HG, Kraemer-Hansen H, Luttges J, Kremer B, Henne-Bruns D. Recurrence of primary biliary cirrhosis after orthotopic liver transplantation. Hepatogastroenterology 50: 322-325 (2003).

• Kurien BT. Affinity purification of autoantibodies from an antigen strip excised from a nitrocellulose protein blot. Methods Mol. Biol. 536: 201-211 (2009).

• Kurien BT, Scofield RH. Multiple nuclear dot antinuclear antibody in patients without primary biliary cirrhosis. Indian J. Biochem. Biophys. 35: 41-47 (1998).

• Kurtovic J, Riordan S. Clinical aspects of primary biliary cirrhosis. Falk Symposium 136. XII FALK LIVER WEEK 2003 (Part I) In Honour of Hans Popper’s 100th Birthday. Cholestatic Liver Diseases: Therapeutical Options and Perspectives. Freiburg (Germany). p. 31-32. October 15-16 (2003). *

• Laadhar L, Zitouni M, Kallel-Sellami M, Bouguerra R, Chaabouni H, Makni S. Spectrum of autoantibodies in Tunisian adult type 1 diabetes mellitus. Ann. N. Y. Acad. Sci. 1107: 356-362 (2007). *

• Labro MT, Andrieu MC, Weber M, Homberg JC. A new pattern of non-organ- and non-species-specific anti-organelle antibody detected by immunofluorescence: the mitochondrial antibody number 5. Clin. Exp. Immunol. 31: 357-366 (1978). *

• Lacerda MA, Ludwig J, Dickson ER, Jorgensen RA, Lindor KD. Antimitochondrial antibody-negative primary biliary cirrhosis. Am. J. Gastroenterol. 90: 247-249 (1995).

• Ladefoged K, Andersen P, Jorgensen J. Autoantibodies and serum immunoglobulins in chronic liver diseases. Acta Med. Scand. 205: 103-109 (1979).

• Lakatos LP, Bajnok E, Hegedus D, Toth T, Lakatos P, Szalay F. Vitamin D receptor, oestrogen receptor-alpha gene and interleukin-1 receptor antagonist gene polymorphisms in Hungarian patients with primary biliary cirrhosis. Eur. J. Gastroenterol. Hepatol. 14: 733-740 (2002).

• Lakatos PL, Bajnok E, Tornai I, Folhoffer A, Horvath A, Lakatos P, Habior A, Szalay F. Insulin-like growth factor I gene microsatellite repeat, collagen type Ialpha1 gene Sp1 polymorphism, and bone disease in primary biliary cirrhosis. Eur. J. Gastroenterol. Hepatol. 16: 753-759 (2004).

• Lamhonwah AM, Ackerley C, Onizuka R, Tilups A, Lamhonwah D, Chung C, Tao KS, Tellier R, Tein I. Epitope shared by functional variant of organic cation/carnitine transporter, OCTN1, Campylobacter jejuni and Mycobacterium paratuberculosis may underlie susceptibility to Crohn's disease at 5q31. Biochem. Biophys. Res. Commun. 337: 1165-1175 (2005). *

• Lamhonwah AM, Tein I. Novel localization of OCTN1, an organic cation/carnitine transporter, to mammalian mitochondria. Biochem. Biophys. Res. Commun. 345: 1315-1325 (2006). *

• Lang P, Klein R, Becker EW, Berg PA. Distribution of the PBC-specific-(M2) and the naturally-occurring mitochondrial antigen-(NOMAg) systems in plants. Clin. Exp. Immunol. 90: 509-516 (1992). *

• Lassoued K, Guilly MN, Andre C, Paintrand M, Dhumeaux D, Danon F, Brouet JC, Courvalin JC. Autoantibodies to 200 kD polypeptide(s) of the nuclear envelope: a new serologic marker of primary biliary cirrhosis. Clin. Exp. Immunol. 74: 283-288 (1988). *

• Laurent A, Nicco C, Tran Van Nhieu J, Borderie D, Chereau C, Conti F, Jaffray P, Soubrane O, Calmus Y, Weill B, Batteux F. Pivotal role of superoxide anion and beneficial effect of antioxidant molecules in murine steatohepatitis. Hepatology 39: 1277-1285 (2004). *

• Laurin JM, Lindor KD. Primary biliary cirrhosis. Dig. Dis. 12: 331-350 (1994).

• Lauritsen K, Diederichsen H. Arthritis in patients with antimitochondrial antibodies. Scand. J. Rheumatol. 12: 331-335 (1983).

• Lawrence JW, Li Y, Chen S, DeLuca JG, Berger JP, Umbenhauer DR, Moller DE, Zhou G. Differential gene regulation in human versus rodent hepatocytes by peroxisome proliferator-activated receptor (PPAR) alpha. J. Biol. Chem. 276: 31521-31527 (2001). *

• Lazaridis KN, Juran BD, Boe GM, Slusser JP, de Andrade M, Homburger HA, Ghosh K, Dickson ER, Lindor KD, Petersen GM. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis. Hepatology 46: 785-792 (2007). *

• Lazaridis KN, Lindor KD. Primary biliary cirrhosis. Curr. Treat. Options Gastroenterol. 2: 473-480 (1999).

• Lazaridis KN, Talwalkar JA. Clinical epidemiology of primary biliary cirrhosis: incidence, prevalence, and impact of therapy. J. Clin. Gastroenterol. 41: 494-500 (2007).

• Lee J, Chang KH, Park CS, Lee CH, Song CH, Park YN, Park C, Lee SK. A case of autoimmune cholangitis associated with Sjogren's syndrome and arthropathy. J. Korean Med. Sci. 13: 85-88 (1998). *

• Lee M, Bae MA. Docosahexaenoic acid induces apoptosis in CYP2E1-containing HepG2 cells by activating the c-Jun N-terminal protein kinase related mitochondrial damage. J. Nutr. Biochem. 18: 348-354 (2007). *

• Lee S, Ansari AA, Cha S. Isolation of human anti-branched chain alpha-oxo acid dehydrogenase-E2 recombinant antibodies by Ig repertoire cloning in idiopathic dilated cardiomyopathy. Mol. Cells 9: 25-30 (1999).

• Lee SM, Ko SH, Park JE, Cha SH. Expression of a recombinant branched chain alpha-oxo acid dehydrogenase complex E2 (BCOADC-E2) in insect cells and its immunoreactivity to autoimmune sera. Exp. Mol. Med. 30: 65-71 (1998). *

• Lee TY, Chen FY, Chang HH, Lin HC. The effect of capillarisin on glycochenodeoxycholic acid-induced apoptosis and heme oxygenase-1 in rat primary hepatocytes. Mol. Cell. Biochem. 325: 53-59 (2009). *

• Lee WM, Shelton LL, Galbraith RM. Antibodies to hepatocyte membrane antigens in chronic liver disease: detection by immunofluorescence after Bouin's fixation. J. Histochem. Cytochem. 31: 1246-1249 (1983).

• Lee YM, Kaplan MM. Efficacy of colchicine in patients with primary biliary cirrhosis poorly responsive to ursodiol and methotrexate. Am. J. Gastroenterol. 98: 205-208 (2003). *

• Lefkowitch JH. Hepatobiliary pathology. Curr. Opin. Gastroenterol. 18: 290-298 (2002).

• Lefkowitch JH. Hepatobiliary pathology. Curr. Opin. Gastroenterol. 19: 185-193 (2003).

• Lefkowitch JH. Hepatobiliary pathology. Curr. Opin. Gastroenterol. 23: 221-231 (2007).

• Lefkowitch JH. Hepatobiliary pathology. Curr. Opin. Gastroenterol. 20: 188-197 (2004).

• Lei HY, Chang CP. Induction of autophagy by concanavalin A and its application in anti-tumor therapy. Autophagy 3: 402-404 (2007).

• Lenhard JM, Kliewer SA, Paulik MA, Plunket KD, Lehmann JM, Weiel JE. Effects of troglitazone and metformin on glucose and lipid metabolism: alterations of two distinct molecular pathways. Biochem. Pharmacol. 54: 801-808 (1997). *

• Leo MA, Aleynik SI, Aleynik MK, Lieber CS. beta-Carotene beadlets potentiate hepatotoxicity of alcohol. Am. J. Clin. Nutr. 66: 1461-1469 (1997). *

• Leung PS, Cha S, Joplin RE, Galperin C, Van de Water J, Ansari AA, Coppel RL, Schatz PJ, Cwirla S, Fabris LE, Neuberger JM, Gershwin ME. Inhibition of PDC-E2 human combinatorial autoantibodies by peptide mimotopes. J. Autoimmun. 9: 785-793 (1996). *

• Leung PS, Chuang DT, Wynn RM, Cha S, Danner DJ, Ansari A, Coppel RL, Gershwin ME. Autoantibodies to BCOADC-E2 in patients with primary biliary cirrhosis recognize a conformational epitope. Hepatology 22: 505-513 (1995).

• Leung PS, Coppel RL, Ansari A, Munoz S, Gershwin ME. Antimitochondrial antibodies in primary biliary cirrhosis. Semin. Liver Dis. 17: 61-69 (1997).

• Leung PS, Coppel RL, Gershwin ME. Etiology of primary biliary cirrhosis: the search for the culprit. Semin. Liver Dis. 25: 327-336 (2005).

• Leung PS, Iwayama T, Coppel RL, Gershwin ME. Site-directed mutagenesis of lysine within the immunodominant autoepitope of PDC-E2. Hepatology 12: 1321-1328 (1990).

• Leung PS, Iwayama T, Prindiville T, Chuang DT, Ansari AA, Wynn RM, Dickson R, Coppel R, Gershwin ME. Use of designer recombinant mitochondrial antigens in the diagnosis of primary biliary cirrhosis. Hepatology 15: 367-372 (1992).

• Leung PS, Krams S, Munoz S, Surh CP, Ansari A, Kenny T, Robbins DL, Fung J, Starzl TE, Maddrey W. Characterization and epitope mapping of human monoclonal antibodies to PDC-E2, the immunodominant autoantigen of primary biliary cirrhosis. J. Autoimmun. 5: 703-718 (1992).

• Leung PS, Park O, Tsuneyama K, Kurth MJ, Lam KS, Ansari AA, Coppel RL, Gershwin ME. Induction of primary biliary cirrhosis in guinea pigs following chemical xenobiotic immunization. J. Immunol. 179: 2651-2657 (2007). *

• Leung PS, Quan C, Park O, Van de Water J, Kurth MJ, Nantz MH, Ansari AA, Coppel RL, Lam KS, Gershwin ME. Immunization with a xenobiotic 6-bromohexanoate bovine serum albumin conjugate induces antimitochondrial antibodies. J. Immunol. 170: 5326-5332 (2003). *

• Leung PS, Rossaro L, Davis PA, Park O, Tanaka A, Kikuchi K, Miyakawa H, Norman GL, Lee W, Gershwin ME; Acute Liver Failure Study Group. Antimitochondrial antibodies in acute liver failure: implications for primary biliary cirrhosis. Hepatology 46: 1436-1442 (2007). *

• Leung PS, Van de Water J, Coppel RL, Nakanuma Y, Munoz S, Gershwin ME. Molecular aspects and the pathological basis of primary biliary cirrhosis. J. Autoimmun. 9: 119-128 (1996). *

• Leuschner M, Dietrich CF, You T, Seidl C, Raedle J, Herrmann G, Ackermann H, Leuschner U. Characterisation of patients with primary biliary cirrhosis responding to long term ursodeoxycholic acid treatment. Gut 46: 121-126 (2000). *

• Leuschner M, Holtmeier J, Ackermann H, Leuschner U. The influence of sulindac on patients with primary biliary cirrhosis that responds incompletely to ursodeoxycholic acid: a pilot study. Eur. J. Gastroenterol. Hepatol. 14: 1369-1376 (2002).

• Leuschner M, Maier KP, Schlichting J, Strahl S, Herrmann G, Dahm HH, Ackermann H, Happ J, Leuschner U. Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial. Gastroenterology 117: 918-925 (1999). *

• Leuschner U. Primary biliary cirrhosis - presentation and diagnosis. Clin. Liver Dis. 7: 741-758 (2003).

• Leuschner U, Guldutuna S, Imhof M, Hubner K, Benjaminov A, Leuschner M. Effects of ursodeoxycholic acid after 4 to 12 years of therapy in early and late stages of primary biliary cirrhosis. J. Hepatol. 21: 624-633 (1994).

• Levinson JR, Gordon SC. Liver diseases in the elderly. Clin. Geriatr. Med. 7: 371-385 (1991).

• Levy C, Lindor KD. Current management of primary biliary cirrhosis and primary sclerosing cholangitis. J. Hepatol. 38: S24-S37 (2003). *

• Levy C, Lindor KD. Treatment options for primary biliary cirrhosis and primary sclerosing cholangitis. Curr. Treat. Options Gastroenterol. 6: 93-103 (2003).

• Lewis ND, Asim M, Barry DP, Singh K, de Sablet T, Boucher JL, Gobert AP, Chaturvedi R, Wilson KT. Arginase II restricts host defense to Helicobacter pylori by attenuating inducible nitric oxide synthase translation in macrophages. J. Immunol. Jan 22 [Epub ahead of print] (2010).

• Lewis RS. Calcium signaling mechanisms in T lymphocytes. Annu. Rev. Immunol. 19: 497-521 (2001). *

• Li CH, Xu PS, Wang CY, Zhang Y, Zou GL. The presence of anti-mitochondrial antibodies in Chinese patients with liver involvement in systemic lupus erythematosus. Rheumatol. Int. 26: 697-703 (2006). *

• Li CH, Xu PS, Wang CY, Zou GL. Increased serum aminotransferases associated with anti-mitochondrial antibodies in systemic lupus erythematosus patients with autoimmune liver disease. Clin. Chim. Acta 365: 135-142 (2006). *

• Li CP, Hwang SJ, Chan CY, Lee FY, Huang YS, Chang FY, Lee SD. Clinical evaluation of primary biliary cirrhosis in Chinese patients without serum anti-mitochondrial antibody. Zhonghua Yi Xue Za Zhi (Taipei) 59: 334-340 (1997).

• Li CP, Tong MJ, Hwang SJ, Luo JC, Co RL, Tsay SH, Chang FY, Lee SD. Autoimmune cholangitis with features of autoimmune hepatitis: successful treatment with immunosuppressive agents and ursodeoxycholic acid. J. Gastroenterol. Hepatol. 15: 95-98 (2000). *

• Li L, Prabhakaran K, Zhang X, Borowitz JL, Isom GE. PPARalpha-mediated up-regulation of uncoupling protein-2 switches cyanide-induced apoptosis to necrosis in primary cortical cells. Toxicol. Sci. 93: 136-145 (2006). *

• Li XD, Sun L, Seth RB, Pineda G, Chen ZJ. Hepatitis C virus protease NS3/4A cleaves mitochondrial antiviral signaling protein off the mitochondria to evade innate immunity Proc. Natl. Acad. Sci. U.S.A. 102: 17717-17722 (2005). *

• Liangpunsakul S, Chalasani N. Treatment of nonalcoholic fatty liver disease. Curr. Treat. Options Gastroenterol. 6: 455-463 (2003).

• Liao S, Bao X, Liu T, Lai S, Li K, Garofalo RP, Casola A. Role of retinoic acid inducible gene-I in human metapneumovirus-induced cellular signalling. J. Gen. Virol. 89: 1978-1986 (2008). *

• Liaskos C, Bogdanos DP, Rigopoulou EI, Dalekos GN. Development of antimitochondrial antibodies in patients with autoimmune hepatitis: art of facts or an artifact? J. Gastroenterol. Hepatol. 22: 454-455 (2007). *

• Lieber CS. Biochemical factors in alcoholic liver disease. Semin. Liver Dis. 13: 136-153 (1993).

• Lieber CS. Hepatic, metabolic and toxic effects of ethanol: 1991 update. Alcohol Clin. Exp. Res. 15: 573-592 (1991). *

• Lin XF, Zhao BX, Chen HZ, Ye XF, Yang CY, Zhou HY, Zhang MQ, Lin SC, Wu Q. RXRalpha acts as a carrier for TR3 nuclear export in a 9-cis retinoic acid-dependent manner in gastric cancer cells. J. Cell Sci. 117: 5609-5621 (2004). *

• Lin ZX, Jiao BW, Che CT, Zuo Z, Mok CF, Zhao M, Ho WK, Tse WP, Lam KY, Fan RQ, Yang ZJ, Cheng CH. Ethyl acetate fraction of the root of Rubia cordifolia L. inhibits keratinocyte proliferation in vitro and promotes keratinocyte differentiation in vivo: potential application for psoriasis treatment. Phytother. Res. Dec 3 [Epub ahead of print] (2009).

• Lindenborn-Fotinos J, Sayers TJ, Berg PA. Mitochondrial antibodies in primary biliary cirrhosis. VI. Association of the complement fixing antigen with a component of the mitochondrial F1-ATPase complex. Clin. Exp. Immunol. 50: 267-274 (1982). *

• Lindgren S, Forsberg B, Eriksson S. Observations during long-term plasma exchange in primary biliary cirrhosis. Scand. J. Gastroenterol. 20: 1124-1126 (1985).

• Lindgren S, Manthorpe R, Eriksson S. Autoimmune liver disease in patients with primary Sjogren's syndrome. J. Hepatol. 20: 354-358 (1994).

• Lindor KD. Primary biliary cirrhosis: questions and promises. Ann. Intern. Med. 126: 733-735 (1997). *

• Lindor KD, Dickson ER, Jorgensen RA, Anderson ML, Wiesner RH, Gores GJ, Lange SM, Rossi SS, Hofmann AF, Baldus WP. The combination of ursodeoxycholic acid and methotrexate for patients with primary biliary cirrhosis: the results of a pilot study. Hepatology 22: 1158-1162 (1995).

• Lindskog M, Gleissman H, Ponthan F, Castro J, Kogner P, Johnsen JI. Neuroblastoma cell death in response to docosahexaenoic acid: sensitization to chemotherapy and arsenic-induced oxidative stress. Int. J. Cancer 118: 2584-2593 (2006). *

• Linhart P, Borner N, Leiss O. What is the onset of primary biliary liver cirrhosis? Z. Gastroenterol. 31 Suppl. 2: 94-95 (1993).

• Lino M, Binaut R, Noel LH, Patey N, Rustin P, Daniel L, Serpaggi J, Varaut A, Vanhille P, Knebelmann B, Grunfeld JP, Fakhouri F. Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis. Am. J. Kidney Dis. 46: e41-e46 (2005).

• Lio D, Scola L, Crivello A, Colonna-Romano G, Candore G, Bonafe M, Cavallone L, Franceschi C, Caruso C. Gender-specific association between -1082 IL-10 promoter polymorphism and longevity. Genes Immun. 3: 30-33 (2008). *

• Liou JY, Ghelani D, Yeh S, Wu KK. Nonsteroidal anti-inflammatory drugs induce colorectal cancer cell apoptosis by suppressing 14-3-3{varepsilon}. Cancer Res. 67: 3185-3191 (2007). *

• Liou JY, Lee S, Ghelani D, Matijevic-Aleksic N, Wu KK. Protection of endothelial survival by peroxisome proliferator-activated receptor-delta mediated 14-3-3 upregulation. Arterioscler. Thromb. Vasc. Biol. 26: 1481-1487 (2006).

• Liou JY, Wu CC, Chen BR, Yen LB, Wu KK. Nonsteroidal anti-inflammatory drugs induced endothelial apoptosis by perturbing PPAR-delta transcriptional pathway. Mol. Pharmacol. 74: 1399-1406 (2008). *

• Liu B, Li Z, Mahesh SP, Pantanelli S, Hwang FS, Siu WO, Nussenblatt RB. Glucocorticoid-induced tumor necrosis factor receptor negatively regulates activation of primary human natural killer (NK) cells by blocking proliferative signals and increasing NK cell apoptosis. J. Biol. Chem. 283: 8202-8210 (2008). *

• Liu B, Shi XH, Zhang FC, Zhang W, Gao LX. Antimitochondrial antibody-negative primary biliary cirrhosis: a subset of primary biliary cirrhosis. Liver Int. 28: 233-239 (2008). *

• Liu C, Crawford JM. Chapter 10. Clinical use of immunopathology techniques in liver diseases. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 123-136 (2007). *

• Liu H, Huang Q, Shi B, Eksarko P, Temkin V, Pope RM. Regulation of Mcl-1 expression in rheumatoid arthritis synovial macrophages. Arthritis Rheum. 54: 3174-3181 (2006). *

• Liu J, Epand RF, Durrant D, Grossman D, Chi NW, Epand RM, Lee RM. Role of phospholipid scramblase 3 in the regulation of tumor necrosis factor-alpha-induced apoptosis. Biochemistry 47: 4518-4529 (2008). *

• Liu W, Li WM, Sun NL. HLA-DQA1, -DQB1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy in Hans of northern China. Ann. Hum. Genet. 69: 382-388 (2005).

• Liu W, Li WM, Yang SS, Gao C, Li SJ, Li Y, Kong YH, Gan RT. Association of HLA class II DRB1, DPA1 and DPB1 polymorphism with genetic susceptibility to idiopathic dilated cardiomyopathy in Chinese Han nationality. Autoimmunity 39: 461-467 (2006).

• Liu Z, Mutlib AE, Wang J, Talaat RE. Liquid chromatography/mass spectrometry determination of endogenous plasma acetyl and palmitoyl carnitines as potential biomarkers of beta-oxidation in mice. Rapid Commun. Mass Spectrom. 22: 3434-3442 (2008). *

• Lleo A, Invernizzi P, Mackay IR, Prince H, Zhong RQ, Gershwin ME. Etiopathogenesis of primary biliary cirrhosis. World J. Gastroenterol. 14: 3328-3337 (2008). *

• Lleo A, Selmi C, Invernizzi P, Podda M, Coppel RL, Mackay IR, Gores GJ, Ansari AA, Van de Water J, Gershwin ME. Apotopes and the biliary specificity of primary biliary cirrhosis. Hepatology 49: 871-879 (2009). *

• Lo AS, Zhu Q, Marasco WA. Intracellular antibodies (intrabodies) and their therapeutic potential. Handb. Exp. Pharmacol. 181: 343-373 (2008).

• Lohr H, Fleischer B, Gerken G, Meyer zum Buschenfelde KH, Manns M. Infiltrating T-cells in primary biliary cirrhosis recognize mitochondrial epitopes. Z. Gastroenterol. 31 Suppl. 2: 99-103 (1993).

• Lohr H, Fleischer B, Gerken G, Yeaman SJ, Meyer zum Buschenfelde KH, Manns M. Autoreactive liver-infiltrating T cells in primary biliary cirrhosis recognize inner mitochondrial epitopes and the pyruvate dehydrogenase complex. J. Hepatol. 18: 322-327 (1993).

• Lohr HF. Autoimmune hepatitis and overlap syndrome: therapy. Schweiz. Rundsch. Med. Prax. 91: 1347-1351 (2002).

• Lohse AW, Reckmann A, Kyriatsoulis A, Gerken G, Meyer zum Buschenfelde KH, Manns M. In vitro secretion of specific antimitochondrial antibodies in primary biliary cirrhosis. J. Hepatol. 16: 165-170 (1992).

• Lohse AW, zum Buschenfelde KH, Franz B, Kanzler S, Gerken G, Dienes HP. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. Hepatology 29: 1078-1084 (1999). *

• Long SA, Quan C, Van de Water J, Nantz MH, Kurth MJ, Barsky D, Colvin ME, Lam KS, Coppel RL, Ansari A, Gershwin ME. Immunoreactivity of organic mimeotopes of the E2 component of pyruvate dehydrogenase: connecting xenobiotics with primary biliary cirrhosis. J. Immunol. 167: 2956-2963 (2001). *

• Long SA, Van de Water J, Gershwin ME. Antimitochondrial antibodies in primary biliary cirrhosis: the role of xenobiotics. Autoimmun. Rev. 1: 37-42 (2002).

• Loria P, Lonardo A, Leonardi F, Fontana C, Carulli L, Verrone AM, Borsatti A, Bertolotti M, Cassani F, Bagni A, Muratori P, Ganazzi D, Bianchi FB, Carulli N. Non-organ-specific autoantibodies in nonalcoholic fatty liver disease: prevalence and correlates. Dig. Dis. Sci. 48: 2173-2181 (2003). *

• Lozano A, Vildosola H, Takano J, Vargas G, Uribe R, Moreno C. Clinical and immunological profile in 22 patients with primary biliary cirrhosis. Rev. Gastroenterol. Peru. 20: 14-24 (2000).

• Lozano F, Pares A, Borche L, Plana M, Gallart T, Rodes J, Vives J. Autoantibodies against nuclear envelope-associated proteins in primary biliary cirrhosis. Hepatology 8: 930-938 (1988).

• Lu JX, Qian SY, Shu X, Li G. The significance of antimitochondrial IgA and IgG in the diagnosis of primary biliary cirrhosis. Zhonghua Gan Zang Bing Za Zhi 17: 905-909 (2009).

• Lu W, Sun G. Clinical characteristics of primary biliary cirrhosis with Sjogren's syndrome of 20 cases. Zhonghua Nei Ke Za Zhi 40: 747-749 (2001).

• Ludwig J, Wiesner RH, LaRusso NF. Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis. J. Hepatol. 7: 193-199 (1988).

• Luettig B, Boeker KH, Schoessler W, Will H, Loges S, Schmidt E, Worman HJ, Gershwin ME, Manns MP. The antinuclear autoantibodies Sp100 and gp210 persist after orthotopic liver transplantation in patients with primary biliary cirrhosis. J. Hepatol. 28: 824-828 (1998). *

• Lugering A, Schmidt M, Lugering N, Pauels HG, Domschke W, Kucharzik T. Infliximab induces apoptosis in monocytes from patients with chronic active Crohn's disease by using a caspase-dependent pathway. Gastroenterology 121: 1145-1157 (2001). *

• Lumini JA, Magalhaes J, Oliveira PJ, Ascensao A. Beneficial effects of exercise on muscle mitochondrial function in diabetes mellitus. Sports Med. 38: 735-750 (2008).

• Mabalirajan U, Dinda AK, Sharma SK, Ghosh B. Esculetin restores mitochondrial dysfunction and reduces allergic asthma features in experimental murine model. J. Immunol. 183: 2059-2067 (2009).

• Macak J. Antibodies against mitochondria and smooth muscle in primary biliary cirrhosis. Cesk. Patol. 12: 175-179 (1976).

• Macdonald P, Palmer J, Kirby JA, Jones DE. Apoptosis as a mechanism for cell surface expression of the autoantigen pyruvate dehydrogenase complex. Clin. Exp. Immunol. 136: 559-567 (2004). *

• Machida K, Cheng KT, Lai CK, Jeng KS, Sung VM, Lai MM. Hepatitis C virus triggers mitochondrial permeability transition with production of reactive oxygen species, leading to DNA damage and STAT3 activation. J. Virol. 80: 7199-7207 (2006). *

• Mackay IR. Autoimmunity and primary biliary cirrhosis. Baillieres Best Pract. Res. Clin. Gastroenterol. 14: 519-533 (2000).

• Mackay IR. Foreword. Contemporary liver immunology and immunopathology: obstacles and opportunities. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 1-14 (2007). *

• Mackay IR, Gershwin ME. Primary biliary cirrhosis: current knowledge, perspectives, and future directions. Semin. Liver Dis. 9: 149-157 (1989).

• Mackay IR, Gershwin ME. Molecular basis of mitochondrial autoreactivity in primary biliary cirrhosis. Immunol. Today 10: 315-318 (1989).

• Mackay IR, Whittingham S, Fida S, Myers M, Ikuno N, Gershwin ME, Rowley MJ. The peculiar autoimmunity of primary biliary cirrhosis. Immunol. Rev. 174: 226-237 (2000). *

• MacQuillan GC, Neuberger J. Liver transplantation for primary biliary cirrhosis. Clin. Liver Dis. 7: 941-956, ix (2003).

• Maczurek A, Hager K, Kenklies M, Sharman M, Martins R, Engel J, Carlson D, Munch G. Lipoic acid as an anti-inflammatory and neuroprotective treatment for Alzheimer's disease. Adv. Drug Deliv. Rev. 60: 1463-1470 (2008).

• Maeda T, Loveland BE, Rowley MJ, Mackay IR. Autoantibody against dihydrolipoamide dehydrogenase, the E3 subunit of the 2-oxoacid dehydrogenase complexes: significance for primary biliary cirrhosis. Hepatology 14: 994-999 (1991).

• Maekawa S, Yano E, Shintani S. A case of rheumatoid arthritis associated with progressive systemic sclerosis and primary biliary cirrhosis in the presence of various autoantibodies. Ryumachi. 32: 515-521 (1992).

• Magnani HN, Alaupovic P. Utilization of the quantitative assay of lipoprotein X in the differential diagnosis of extraphepatic obstructive jaundice and intrahepatic diseases. Gastroenterology 71: 87-93 (1976).

• Maher JJ. What doesn't kill you makes you stronger: how hepatocytes survive prolonged cholestasis. Hepatology 39: 1141-1143 (2004). *

• Makowski L, Noland RC, Koves TR, Xing W, Ilkayeva OR, Muehlbauer MJ, Stevens RD, Muoio DM. Metabolic profiling of PPARalpha-/- mice reveals defects in carnitine and amino acid homeostasis that are partially reversed by oral carnitine supplementation. FASEB J. 23: 586-604 (2009). *

• Malaguarnera M, Guccione N, Musumeci S, Brogna A, Motta M, Di Fazio I. Intravenous immunoglobulin plus interferon-alpha in autoimmune hepatitis C. BioDrugs 18: 63-70 (2004).

• Mallard K, Jones DB, Richmond J, McGill M, Foulis AK. Expression of the human heat shock protein 60 in thyroid, pancreatic, hepatic and adrenal autoimmunity. J. Autoimmun. 9: 89-96 (1996).

• Malmborg AC, Shultz DB, Luton F, Mostov KE, Richly E, Leung PS, Benson GD, Ansari AA, Coppel RL, Gershwin ME, Van de Water J. Penetration and co-localization in MDCK cell mitochondria of IgA derived from patients with primary biliary cirrhosis. J. Autoimmun. 11: 573-580 (1998). *

• Manns M, Gerken G, Kyriatsoulis A, Trautwein C, Reske K, Meyer Zum Buschenfelde KH. Two different subtypes of antimitochondrial antibodies are associated with primary biliary cirrhosis: identification and characterization by radioimmunoassay and immunoblotting. Hepatology 7: 893-899 (1987).

• Manns M, Gerken G, Trautwein C, Meuer M, Porally T, Dienes HP, Meyer zum Buschenfelde KH. Characterization of primary biliary cirrhosis (PBC) specific mitochondrial determinants by immunoblotting. Dtsch. Z. Verdau. Stoffwechselkr. 47: 59-66 (1987).

• Manns M, Meyer zum Buschenfelde KH. A mitochondrial antigen-antibody system in cholestatic liver disease detected by radioimmunoassay. Hepatology 2: 1-7 (1982).

• Manns M, Meyer zum Buschenfelde KH, Arnold W. The diagnostic value of immunologic findings in the differentiation of chronic liver diseases. Leber. Magen. Darm. 18: 290-292, 295, 298 (1988).

• Manns M, Meyer zum Buschenfelde KH, Slusarczyk J, Dienes HP. Detection of liver-kidney microsomal autoantibodies by radioimmunoassay and their relation to anti-mitochondrial antibodies in inflammatory liver diseases. Clin. Exp. Immunol. 57: 600-608 (1984). *

• Manns MP. Recent developments in autoimmune liver diseases. J. Gastroenterol. Hepatol. 12: S256-S271 (1997).

• Manns MP. Round table: classification of autoimmune liver diseases and diagnostic criteria. Subgroups of autoimmune hepatitis are important. Falk Symposium 142: GASTROENTEROLOGY WEEK FREIBURG 2004 (Part I) AUTOIMMUNE LIVER DISEASE, Freiburg (Germany), October 12 - 13, 2004, pp. 56-57 (2004). *

• Manns MP, Nakamura RM. Autoimmune liver diseases. Clin. Lab. Med. 8: 281-301 (1988).

• Manuel Lucena J, Montes Cano M, Luis Caro J, Respaldiza N, Alvarez A, Sanchez-Roman J, Nunez-Roldán A, Wichmann I. Comparison of two ELISA assays for anti-sp100 determination. Ann. N. Y. Acad. Sci. 1109: 203-211 (2007). *

• Marchenko S, Chernyavsky AI, Arredondo J, Gindi V, Grando SA. Antimitochondrial autoantibodies in pemphigus vulgaris: a missing link in disease pathophysiology. J. Biol. Chem. Dec 14 [Epub ahead of print] (2009). *

• Marcos M, Alvarez F, Brito-Zeron P, Bove A, Perez-De-Lis M, Diaz C, Sanchez-Tapias JM, Ramos-Casals M. Chronic hepatitis B virus infection in Sjogren's syndrome: prevalence and clinical significance in 603 patients. Autoimmun. Rev. 8: 616-620 (2009).

• Marpeau L, Benifla JL, Chazouilleres O, Larue L, Pigne A, Poupon R, Barrat J. Primary biliary cirrhosis and pregnancy. Apropos of a clinical case. Review of the literature. J. Gynecol. Obstet. Biol. Reprod. (Paris) 20: 805-807 (1991).

• Marriott JB, Oliveira DB. Antimitochondrial autoantibodies in anti-glomerular basement membrane disease. Clin. Exp. Immunol. 93: 259-264 (1993). *

• Marshall WS, Watters KD, Hovdestad LR, Cozzi RR, Katoh F. CFTR Cl- channel functional regulation by phosphorylation of focal adhesion kinase at tyrosine 407 in osmosensitive ion transporting mitochondria rich cells of euryhaline killifish. J. Exp. Biol. 212: 2365-2377 (2009). *

• Martin KR, Failla ML, Smith JC Jr. Beta-carotene and lutein protect HepG2 human liver cells against oxidant-induced damage. J. Nutr. 126: 2098-2106 (1996). *

• Martinez S, Berrios S, Fernandez-Donoso R, Brahm J, Cuchacovich M. Antimitochondrial antibody detection by indirect immunofluorescence on mouse sperms. Eur. J. Histochem. 40: 315-322 (1996).

• Martinez S, Berrios S, Morales M, Cuchacovic M, Brahm J, Fernandez-Donoso R. A new substrate for the detection of antimitochondrial antibodies in human serum. Rev. Med. Chil. 122: 889-899 (1994).

• Marucci L, Ugili L, Macarri G, Feliciangeli G, Bendia E, Jezequel AM, Orlandi F, Benedetti A. Primary biliary cirrhosis: modalities of injury and death in biliary epithelium. Dig. Liver Dis. 33: 576-583 (2001).

• Masanaga T, Watanabe Y, Van de Water J, Leung PS, Nakanishi T, Kajiyama G, Ruebner BH, Coppel RL, Gershwin ME. Induction and persistence of immune-mediated cholangiohepatitis in neonatally thymectomized mice. Clin. Immunol. Immunopathol. 89: 141-149 (1998). *

• Mason A. Viral induction of type 2 diabetes and autoimmune liver disease. J. Nutr. 131: 2805S-2808S (2001). *

• Mason A, Nair S. Primary biliary cirrhosis: new thoughts on pathophysiology and treatment. Curr. Gastroenterol. Rep. 4: 45-51 (2002).

• Mason A, Xu L, Shen Z, Fodera B, Joplin R, Neuberger J, O'Donnell B. Patients with primary biliary cirrhosis make anti-viral and anti-mitochondrial antibodies to mouse mammary tumor virus. Gastroenterology 127: 1863-1864 (2004). *

• Mason AL. An autoimmune biliary disease mouse model for primary biliary cirrhosis: something for everyone. Hepatology 44: 1047-1050 (2006). *

• Mason AL, Farr GH, Xu L, Hubscher SG, Neuberger JM. Pilot studies of single and combination antiretroviral therapy in patients with primary biliary cirrhosis. Am. J. Gastroenterol. 99: 2348-2355 (2004). *

• Mason AL, Xu L, Guo L, Munoz S, Jaspan JB, Bryer-Ash M, Cao Y, Sander DM, Shoenfeld Y, Ahmed A, Van de Water J, Gershwin ME, Garry RF. Detection of retroviral antibodies in primary biliary cirrhosis and other idiopathic biliary disorders. Lancet 351: 1620-1624 (1998). *

• Masubuchi Y. Metabolic and non-metabolic factors determining troglitazone hepatotoxicity: a review. Drug Metab. Pharmacokinet. 21: 347-356 (2006). *

• Masuda J, Omagar K, Miyakawa H, Hazama H, Ohba K, Kinoshita H, Matsuo I, Isomoto H, Murata I, Kohno S. Clinical significance of positive immunoblotting but negative immunofluorescence for antimitochondrial antibodies in patients with liver diseases other than primary biliary cirrhosis. Autoimmunity 35: 135-141 (2002).

• Masuda J, Omagari K, Matsuo I, Kinoshita H, Sakimura K, Hazama H, Ohba K, Isomoto H, Murase K, Murata I, Kohno S. Changes in titers of antimitochondrial and antinuclear antibodies during the course of primary biliary cirrhosis. J. Gastroenterol. Hepatol. 16: 239-243 (2001). *

• Masumoto T, Ninomiya T, Michitaka K, Horiike N, Yamamoto K, Akbar SM, Abe M, Onji M. Three patients with autoimmune cholangiopathy treated with prednisolone. J. Gastroenterol. 33: 909-913 (1998). *

• Mathieu J, Flexor M, Lanotte M, Besancon F. A PARP-1/JNK1 cascade participates in the synergistic apoptotic effect of TNFalpha and all-trans retinoic acid in APL cells. Oncogene 27: 3361-3370 (2008). *

• Mato TK, Davis PA, Odin JA, Coppel RL, Gershwin ME. Sidechain biology and the immunogenicity of PDC-E2, the major autoantigen of primary biliary cirrhosis. Hepatology 40: 1241-1248 (2004). *

• Matos M, Capozzolo N, Golindano C, Gonzalez JC, Guirola E, Ramirez R, Machado I. Humoral immunologic changes in inflammatory colonic diseases. G. E. N. 47: 129-135 (1993).

• Matsuda Y, Amuro Y, Hada T, Higashino K, Ueki N, Fujikura M, Tonomura A, Yamamoto T, Yamaguchi K, Shimomura S, et al. Aspartate aminotransferase-linked immunoglobulin complexes in serum of a patient with primary biliary cirrhosis. J. Gastroenterol. 29: 218-222 (1994).

• Matsui M, Nakamura M, Ishibashi H, Koike K, Kudo J, Niho Y. Human monoclonal antibodies from a patient with primary biliary cirrhosis that recognize two distinct autoepitopes in the E2 component of the pyruvate dehydrogenase complex. Hepatology 18: 1069-1077 (1993).

• Matsumoto T, Kobayashi S, Shimizu H, Nakajima M, Watanabe S, Kitami N, Sato N, Abe H, Aoki Y, Hoshi T, Hashimoto H. The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver. Liver 20: 366-373 (2000).

• Matsumura S, Van De Water J, Kita H, Coppel RL, Tsuji T, Yamamoto K, Ansari AA, Gershwin ME. Contribution to antimitochondrial antibody production: cleavage of pyruvate dehydrogenase complex-E2 by apoptosis-related proteases. Hepatology 35: 14-22 (2002). *

• Matsumura S, Van De Water J, Leung P, Odin JA, Yamamoto K, Gores GJ, Mostov K, Ansari AA, Coppel RL, Shiratori Y, Gershwin ME. Caspase induction by IgA antimitochondrial antibody: IgA-mediated biliary injury in primary biliary cirrhosis. Hepatology 39: 1415-1422 (2004). *

• Matsuzaki Y, Tanaka N, Osuga T, Aikawa T, Shoda J, Doi M, Nakano M. Improvement of biliary enzyme levels and itching as a result of long-term administration of ursodeoxycholic acid in primary biliary cirrhosis. Am. J. Gastroenterol. 85: 15-23 (1990).

• Mattalia A, Luttig B, Rosina F, Leung PS, Van de Water J, Bauducci M, Ciancio A, Boker KH, Worman H, Cooper RL, Manns M, Ansari A, Rizzetto M, Gershwin ME. Persistence of autoantibodies against recombinant mitochondrial and nuclear pore proteins after orthotopic liver transplantation for primary biliary cirrhosis. J. Autoimmun. 10: 491-497 (1997). *

• Mattalia A, Quaranta S, Leung PS, Bauducci M, Van de Water J, Calvo PL, Danielle F, Rizzetto M, Ansari A, Coppel RL, Rosina F, Gershwin ME. Characterization of antimitochondrial antibodies in healthy adults. Hepatology 27: 656-661 (1998). *

• Mattner J, Savage PB, Leung P, Oertelt SS, Wang V, Trivedi O, Scanlon ST, Pendem K, Teyton L, Hart J, Ridgway WM, Wicker LS, Gershwin ME, Bendelac A. Liver autoimmunity triggered by microbial activation of natural killer T cells. Cell Host Microbe 3: 304-315 (2008). *

• Mayo MJ, Parkes J, Adams-Huet B, Combes B, Mills AS, Markin RS, Rubin R, Wheeler D, Contos M, West AB, Saldana S, Getachew Y, Butsch R, Luketic V, Peters M, Di Bisceglie A, Bass N, Lake J, Boyer T, Martinez E, Boyer J, Garcia-Tsao G, Barnes D, Rosenberg W. Prediction of clinical outcomes in primary biliary cirrhosis by serum enhanced liver fibrosis assay. Hepatology 48: 1549-1557 (2008). *

• McCarty MF, Barroso-Aranda J, Contreras F. The "rejuvenatory" impact of lipoic acid on mitochondrial function in aging rats may reflect induction and activation of PPAR-gamma coactivator-1alpha. Med. Hypotheses 72: 29-33 (2009).

• McFarlane IG. Autoimmune liver diseases. Scand. J. Clin. Lab. Invest. 235: 53-60 (2001).

• McHugh NJ, James IE, Fairburn K, Maddison PJ. Autoantibodies to mitochondrial and centromere antigens in primary biliary cirrhosis and systemic sclerosis. Clin. Exp. Immunol. 81: 244-249 (1990). *

• McIntyre N. Sheila: Professor Dame Sheila Sherlock (1918-2001). Hepatology 35: 507-509 (2002). *

• McMillan SA, Alderdice JM, McKee CM, Dawson AT, Callender ME, Fulton TT, Haire M. Diversity of autoantibodies in patients with antimitochondrial antibody and their diagnostic value. J. Clin. Pathol. 40: 232-236 (1987). *

• Meek F, Khoury EL, Doniach D, Baum H. Mitochondrial antibodies in chronic liver diseases and connective tissue disorders: further characterization of the autoantigens. Clin. Exp. Immunol. 41: 43-54 (1980). *

• Meek FL. A direct binding assay for mitochondrial autoantibodies. J. Immunol. Methods 51: 109-118 (1982).

• Mehta K, Van Thiel DH, Shah N, Mobarhan S. Nonalcoholic fatty liver disease: pathogenesis and the role of antioxidants. Nutr. Rev. 60: 289-293 (2002).

• Melegh B, Skuta G, Pajor L, Hegedus G, Sumegi B. Autoantibodies against subunits of pyruvate dehydrogenase and citrate synthase in a case of paediatric biliary cirrhosis. Gut 42: 753-756 (1998). *

• Meliconi R, Stancari MV, Garagnani M, Baraldini M, Stefanini GF, Miglio F, Gasbarrini G. Occurrence and significance of IgG liver membrane autoantibodies (LMA) in chronic liver diseases of different aetiology. Clin. Exp. Immunol. 51: 565-571 (1983). *

• Mellier G, Huang S, Shenoy K, Pervaiz S. TRAILing death in cancer. Mol. Aspects Med. Dec 5 [Epub ahead of print] (2009).

• Melnik BC, Schmitz G, Zouboulis CC. Anti-acne agents attenuate FGFR2 signal transduction in acne. J. Invest. Dermatol. 129: 1868-1877 (2009). *

• Mendel-Hartvig I, Frostell A, Nelson BD. Primary biliary cirrhosis: assessment of the quantitative importance of the adenine nucleotide translocator protein as a major mitochondrial antigen. Clin. Exp. Immunol. 66: 399-405 (1986). *

• Mendel-Hartvig I, Frostell A, Totterman TH. Primary biliary cirrhosis: antigenic specificity of IgM-type mitochondrial antibodies analyzed by immunoblotting and ELISA. Int. Arch. Allergy Appl. Immunol. 83: 265-270 (1987).

• Mendel-Hartvig I, Nelson BD, Loof L, Totterman TH. Primary biliary cirrhosis: further biochemical and immunological characterization of mitochondrial antigens. Clin. Exp. Immunol. 62: 371-379 (1985). *

• Mendes F, Lindor KD. Antimitochondrial antibody-negative primary biliary cirrhosis. Gastroenterol. Clin. North Am. 37: 479-484 (2008).

• Mendez-Sanchez N, King-Martínez AC, Ramos MH, Pichardo-Bahena R, Uribe M. The Amerindian's genes in the Mexican population are associated with development of gallstone disease. Am. J. Gastroenterol. 99: 2166-2170 (2004). *

• Metcalf JV, Bhopal RS, Gray J, Howel D, James OF. Incidence and prevalence of primary biliary cirrhosis in the city of Newcastle upon Tyne, England. Int. J. Epidemiol. 26: 830-836 (1997). *

• Metcalf JV, Howel D, James OF, Bhopal R. Primary biliary cirrhosis: epidemiology helping the clinician. BMJ 312: 1181-1182 (1996). *

• Metcalf JV, Mitchison HC, Palmer JM, Jones DE, Bassendine MF, James OF. Natural history of early primary biliary cirrhosis. Lancet 348: 1399-1402 (1996). *

• Meyer zum Buschenfelde KH. Immunology of liver diseases. Schweiz. Med. Wochenschr. 117: 1065-1075 (1987).

• Mezhybovska M, Wikstrom K, Ohd JF, Sjolander A. The inflammatory mediator leukotriene D4 induces beta-catenin signaling and its association with anti-apoptotic Bcl-2 in intestinal epithelial cells. J. Biol. Chem. 281: 6776-6784 (2006). *

• Michel V, Bakovic M. The solute carrier 44A1 is a mitochondrial protein and mediates choline transport. FASEB J. 23: 2749-2758 (2009). *

• Michieletti P, Wanless IR, Katz A, Scheuer PJ, Yeaman SJ, Bassendine MF, Palmer JM, Heathcote EJ. Antimitochondrial antibody negative primary biliary cirrhosis: a distinct syndrome of autoimmune cholangitis. Gut 35: 260-265 (1994). *

• Migliaccio C, Nishio A, Van de Water J, Ansari AA, Leung PS, Nakanuma Y, Coppel RL, Gershwin ME. Monoclonal antibodies to mitochondrial E2 components define autoepitopes in primary biliary cirrhosis. J. Immunol. 161: 5157-5163 (1998). *

• Migliaccio C, Van de Water J, Ansari AA, Kaplan MM, Coppel RL, Lam KS, Thompson RK, Stevenson F, Gershwin ME. Heterogeneous response of antimitochondrial autoantibodies and bile duct apical staining monoclonal antibodies to pyruvate dehydrogenase complex E2: the molecule versus the mimic. Hepatology 33: 792-801 (2001). *

• Miki K, Xu M, Gupta A, Ba Y, Tan Y, Al-Refaie W, Bouvet M, Makuuchi M, Moossa AR, Hoffman RM. Methioninase cancer gene therapy with selenomethionine as suicide prodrug substrate. Cancer Res. 61: 6805-6810 (2001). *

• Milkiewicz P, Buwaneswaran H, Coltescu C, Shums Z, Norman GL, Heathcote EJ. Value of autoantibody analysis in the differential diagnosis of chronic cholestatic liver disease. Clin. Gastroenterol. Hepatol. 7: 1355-1360 (2009).

• Milkiewicz P, Pache I, Buwaneswaran H, Liu X, Coltescu C, Heathcote EJ, Siminovitch KA. The PTPN22 1858T variant is not associated with primary biliary cirrhosis. Tissue Antigens 67: 434-437 (2006). *

• Miller LC, Sharma A, McKusick AF, Tassoni JP, Dinarello CA, Kaplan MM. Synthesis of interleukin-1 beta in primary biliary cirrhosis: relationship to treatment with methotrexate or colchicine and disease progression. Hepatology 22: 518-524 (1995).

• Milne JC, Lambert PD, Schenk S, Carney DP, Smith JJ, Gagne DJ, Jin L, Boss O, Perni RB, Vu CB, Bemis JE, Xie R, Disch JS, Ng PY, Nunes JJ, Lynch AV, Yang H, Galonek H, Israelian K, Choy W, Iffland A, Lavu S, Medvedik O, Sinclair DA, Olefsky JM, et al. Small molecule activators of SIRT1 as therapeutics for the treatment of type 2 diabetes. Nature 450: 712-716 (2007). *

• Min JK, Han NI, Kim JA, Lee YS, Cho CS, Kim HY. A case of cholestatic autoimmune hepatitis and acute liver failure: an unusual hepatic manifestation of mixed connective tissue disease and Sjogren's syndrome. J. Korean Med. Sci. 16: 512-515 (2001). *

• Minuk GY, Boyd ND, Matheson DS, Fritzler MJ, Green BJ. Serum immunoglobulin E levels in patients with primary biliary cirrhosis. J. Allergy Clin. Immunol. 83: 462-466 (1989).

• Minz RW, Chhabra S, Aggarwal R, Das A, Saikia B, Chawla YK. Incipient primary biliary cirrhosis/autoimmune hepatitis overlap or hepatitic form of primary biliary cirrhosis: a case report. Cases J. 2: 7491 (2009). *

• Mishima S, Omagari K, Ohba K, Kadokawa Y, Masuda J, Mishima R, Kinoshita H, Hayashida K, Isomoto H, Shikuwa S, Mizuta Y, Kohno S. Clinical implications of antimitochondrial antibodies in type 1 autoimmune hepatitis: a longitudinal study. Hepatogastroenterology 55: 221-227 (2008).

• Mitchison HC, Bassendine MF, Hendrick A, Bennett MK, Bird G, Watson AJ, James OF. Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis? Hepatology 6: 1279-1284 (1986).

• Mitchison HC, Palmer JM, Bassendine MF, Watson AJ, Record CO, James OF. A controlled trial of prednisolone treatment in primary biliary cirrhosis. Three-year results. J. Hepatol. 15: 336-344 (1992).

• Mitry E, Algayres JP, Bili H, Coutant G, Gros P, Nizou JY, Castagne C, Vergeau B, Daly JP. Autoimmune cholangitis successfully treated with corticotherapy. One case. Gastroenterol. Clin. Biol. 22: 94-97 (1998).

• Mitsui H, Miyauchi E, Miyahara J, Wada K, Yamakawa M, Kawata S. A case of primary biliary cirrhosis accompanied with fibrinogen storage disease. Pathol. Res. Pract. 201: 341-345 (2005).

• Miyachi K, Gupta RC, Dickson ER, Tan EM. Precipitating antibodies to mitochondrial antigens in patients with primary biliary cirrhosis. Clin. Exp. Immunol. 39: 599-606 (1980). *

• Miyachi K, Hankins R, Ihara M, Miyamoto A, Okano T, Iwai M, Mikoshiba K, Fritzler MJ. A case of limited cutaneous systemic sclerosis developing anti-mitochondria antibody positive primary biliary cirrhosis after acute myocardial infarction. Clin. Rheumatol. 26: 1571-1574 (2007).

• Miyachi K, Hosaka H, Nakamura N, Miyakawa H, Mimori T, Shibata M, Matsushima S, Chinoh H, Horigome T, Hankins RW, Zhang M, Fritzler MJ. Anti-p97/VCP antibodies: an autoantibody marker for a subset of primary biliary cirrhosis patients with milder disease? Scand. J. Immunol. 63: 376-382 (2006). *

• Miyachi K, Matsushima H, Hankins RW, Hirakata M, Mimori T, Hosaka H, Amagasaki Y, Miyakawa H, Kako M, Shibata M, Onozuka Y, Ueno U. A novel antibody directed against a three-dimensional configuration of a 95-kDa protein in patients with autoimmune hepatic diseases. Scand. J. Immunol. 47: 63-68 (1998). *

• Miyachi K, Miyakawa H, Oda M, Horigome T, Fritzler M. Primary biliary cirrhosis and autoantibodies. Nihon Rinsho Meneki Gakkai Kaishi 31: 47-55 (2008).

• Miyachi K, Shibata M, Hasegawa C, Onozuka Y, Fritzler MJ. A case of primary biliary cirrhosis patient with anti-p97/VCP antibodies presenting a mild clinical course. Rinsho Byori 53: 19-23 (2005).

• Miyachi K, Watanabe S, Yamashiki M, Hiwatashi T, Ichida F. Precipitating antimitochondrial antibodies in Japanese patients with primary biliary cirrhosis. Am. J. Gastroenterol. 79: 704-709 (1984).

• Miyakawa H, Abe K, Kitazawa E, Kikuchi K, Fujikawa H, Matsushita M, Kawaguchi N, Kako M. Detection of anti-branched chain 2-oxo acid dehydrogenase complex (BCOADC)-E2 antibody in primary biliary cirrhosis by ELISA using recombinant fusion protein. Autoimmunity 30: 11-20 (1999).

• Miyakawa H, Kawaguchi N, Kikuchi K, Fujikawa H, Kitazawa E, Matsushita M. Definition of antigen specificity for antimitochondrial proteins detected by Western blotting using native mitochondrial proteins in primary biliary cirrhosis. Hepatol. Res. 21: 101-107 (2001).

• Miyakawa H, Kawaguchi N, Kikuchi K, Kitazawa E, Kawashima Y, Yajima R, Itoh Y. False positive reaction in ELISA for IgM class anti-M2 antibody and its prevention. Hepatol. Res. 20: 279-287 (2001).

• Miyakawa H, Kitazawa E, Fujikawa H, Kikuchi K, Abe K, Kawaguchi N, Kako M. Analysis of two major anti-M2 antibodies (anti-PDC-E2/anti-BCOADC-E2) in primary biliary cirrhosis: relationship to titers of immunofluorescent anti-mitochondrial antibody. Hepatol. Res. 18: 1-9 (2000).

• Miyakawa H, Tanaka A, Kikuchi K, Matsushita M, Kitazawa E, Kawaguchi N, Fujikawa H, Gershwin ME. Detection of antimitochondrial autoantibodies in immunofluorescent AMA-negative patients with primary biliary cirrhosis using recombinant autoantigens. Hepatology 34: 243-248 (2001). *

• Miyakawa H, Tanaka A, Selmi C, Hosoya N, Mataki N, Kikuchi K, Kato T, Arai J, Goto T, Gershwin ME. Serum reactivity against bacterial pyruvate dehydrogenase: increasing the specificity of anti-mitochondrial antibodies for the diagnosis of primary biliary cirrhosis. Clin. Dev. Immunol. 13: 289-294 (2006). *

• Miyamoto T, Maeda T, Onishi S, Yamamoto Y. T cell autoreactivity against a 28 kD biliary protein (B1-p28) in primary biliary cirrhosis. J. Hepatol. 22: 423-430 (1995). *

• Mizukami Y, Ohhira M, Matsumoto A, Murazumi Y, Murazumi K, Ohta H, Ohhira M, Ono M, Miyake T, Maekawa I, Kohgo Y. Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura. J. Gastroenterol. 31: 284-288 (1996).

• Mizuki M, Tagawa S, Shibano M, Okamoto Y, Nojima J, Sakata K, Takatsuki K, Kawata S, Hashimoto M, Hashimoto K, et al. A HTLV-I carrier who showed various symptoms and antibodies of autoimmune diseases. Intern. Med. 32: 449-454 (1993). *

• Mobacken H, Nilsson LA, Olsson R, Sloberg K. Incidence of liver disease in chronic lichen planus of the mouth. Acta Derm. Venereol. 64: 70-73 (1984).

• Moll UM, Marchenko N, Zhang XK. p53 and Nur77/TR3 - transcription factors that directly target mitochondria for cell death induction. Oncogene 25: 4725-4743 (2006). *

• Montano-Loza AJ, Carpenter HA, Czaja AJ. Frequency, behavior, and prognostic implications of antimitochondrial antibodies in type 1 autoimmune hepatitis. J. Clin. Gastroenterol. 42: 1047-1053 (2008).

• Moon H, Noh J, Hur M, Yun Y, Lee CH, Kwon SY. High prevalence of autoantibodies in hepatitis A infection: the impact on laboratory profiles. J. Clin. Pathol. 62: 786-788 (2009).

• Moran S, Rodriguez-Leal G, Marin-Lopez E, Arista J, Poo JL, Vargas-Vorackova F, Kershenobich D, Uribe M. Primary biliary cirrhosis: clinical features and survival of a Mexican population. Rev. Gastroenterol. Mex. 61: 212-219 (1996).

• Mori T, Ono K, Hakozaki M, Kasukawa R, Kochi H. Autoantibodies of sera from patients with primary biliary cirrhosis recognize the alpha subunit of the decarboxylase component of human branched-chain 2-oxo acid dehydrogenase complex. J. Hepatol. 34: 799-804 (2001). *

• Mori T, Ono K, Hakozaki M, Kochi H. Epitope mapping on E1alpha subunit of pyruvate dehydrogenase complex with autoantibodies of patients with primary biliary cirrhosis. Liver Int. 23: 355-362 (2003). *

• Moritoki Y, Lian ZX, Lindor K, Tuscano J, Tsuneyama K, Zhang W, Ueno Y, Dunn R, Kehry M, Coppel RL, Mackay IR, Gershwin ME. B-cell depletion with anti-CD20 ameliorates autoimmune cholangitis but exacerbates colitis in transforming growth factor-beta receptor II dominant negative mice. Hepatology 50: 1893-1903 (2009). *

• Moritoki Y, Lian ZX, Wulff H, Yang GX, Chuang YH, Lan RY, Ueno Y, Ansari AA, Coppel RL, Mackay IR, Gershwin ME. AMA production in primary biliary cirrhosis is promoted by the TLR9 ligand CpG and suppressed by potassium channel blockers. Hepatology 45: 314-322 (2007). *

• Moritoki Y, Zhang W, Tsuneyama K, Yoshida K, Wakabayashi K, Yang GX, Bowlus C, Ridgway WM, Ueno Y, Ansari AA, Coppel RL, Mackay IR, Flavell RA, Gershwin ME, Lian ZX. B cells suppress the inflammatory response in a mouse model of primary biliary cirrhosis. Gastroenterology 136: 1037-1047 (2009). *

• Morl M, Classen M. Primary sclerosing cholangitis and ulcerative colitis. Acta Hepatogastroenterol. (Stuttg.) 22: 415-419 (1975).

• Moteki S, Amaki S, Nakano E. The association between autoantibodies to enzyme and diseases - with special reference to antibodies to pyruvate dehydrogenase complex (PDH). Rinsho Byori 46: 317-323 (1998).

• Moteki S, Leung PS, Coppel RL, Dickson ER, Kaplan MM, Munoz S, Gershwin ME. Use of a designer triple expression hybrid clone for three different lipoyl domain for the detection of antimitochondrial autoantibodies. Hepatology 24: 97-103 (1996). *

• Moteki S, Leung PS, Dickson ER, Van Thiel DH, Galperin C, Buch T, Alarcon-Segovia D, Kershenobich D, Kawano K, Coppel RL, et al. Epitope mapping and reactivity of autoantibodies to the E2 component of 2-oxoglutarate dehydrogenase complex in primary biliary cirrhosis using recombinant 2-oxoglutarate dehydrogenase complex. Hepatology 23: 436-444 (1996). *

• Mouelhi L, Chaieb M, Sfar I, Debbeche R, Trabelsi S, Gorgi Y, Najjar T. Chronic viral C hepatitis associated with primary biliary cirrhosis. Report of two cases. Rev. Med. Interne. 30: 537-539 (2009).

• Mouritsen S, Demant E, Permin H, Wiik A. High prevalence of anti-mitochondrial antibodies among patients with some well-defined connective tissue diseases. Clin. Exp. Immunol. 66: 68-76 (1986). *

• Mouritsen S, Hoier-Madsen M, Demant EJ, Permin H. Enzyme-linked immunosorbent assay for determination of anti-mitochondrial antibodies. Acta Pathol. Microbiol. Immunol. Scand. [C.] 93: 205-210 (1985).

• Mozsik G, Domotor A, Rumi G, Szekeres G. Gastrointestinal cytoprotection: from basic science to clinical perspectives. Inflammopharmacology 15: 49-60 (2007).

• Mu FT, Alderuccio F, Toh BH, Gershwin ME, Coppel R, Mackay IR. Murine monoclonal antibody to mitochondria reacts with the 72 kD antigen of primary biliary cirrhosis. Clin. Exp. Immunol. 71: 100-106 (1988). *

• Mueller A, Merrell DS, Grimm J, Falkow S. Profiling of microdissected gastric epithelial cells reveals a cell type-specific response to Helicobacter pylori infection. Gastroenterology 127: 1446-1462 (2004). *

• Munoz LE, del Rocio Ayala SR. The clinical laboratory in the diagnosis of autoimmune liver diseases. Rev. Gastroenterol. Mex. 58: 164-169 (1993).

• Munoz LE, Thomas HC, Scheuer PJ, Doniach D, Sherlock S. Is mitochondrial antibody diagnostic of primary biliary cirrhosis? Gut 22: 136-140 (1981). *

• Muratori L, Cassani F, Pappas G, Guidi M, Mele L, Lorenza V, Lenzi M, Bianchi FB, Muratori P. The hepatitic/cholestatic "overlap" syndrome: an Italian experience. Autoimmunity 35: 565-568 (2002).

• Muratori L, Granito A, Muratori P, Pappas G, Bianchi FB. Antimitochondrial antibodies and other antibodies in primary biliary cirrhosis: diagnostic and prognostic value. Clin. Liver Dis. 12: 261-276 (2008).

• Muratori L, Muratori P, Granito A, Ferrari R, Veronesi L, Lenzi M, Bianchi FB. The Western immunoblotting pattern of anti-mitochondrial antibodies is independent of the clinical expression of primary biliary cirrhosis. Dig. Liver Dis. 37: 108-112 (2005).

• Muratori P, Granito A, Pappas G, Muratori L, Lenzi M, Bianchi FB. Autoimmune liver disease 2007. Mol. Aspects Med. 29: 96-102 (2007).

• Muratori P, Granito A, Pappas G, Pendino GM, Quarneti C, Cicola R, Menichella R, Ferri S, Cassani F, Bianchi FB, Lenzi M, Muratori L. The serological profile of the autoimmune hepatitis/primary biliary cirrhosis overlap syndrome. Am. J. Gastroenterol. 104: 1420-1425 (2009). *

• Muratori P, Muratori L, Ferrari R, Cassani F, Bianchi G, Lenzi M, Rodrigo L, Linares A, Fuentes D, Bianchi FB. Characterization and clinical impact of antinuclear antibodies in primary biliary cirrhosis. Am. J. Gastroenterol. 98: 431-437 (2003). *

• Muratori P, Muratori L, Gershwin ME, Czaja AJ, Pappas G, Maccariello S, Granito A, Cassani F, Loria P, Lenzi M, Bianchi FB. 'True' antimitochondrial antibody-negative primary biliary cirrhosis, low sensitivity of the routine assays, or both? Clin. Exp. Immunol. 135: 154-158 (2004). *

• Murdaca G, Colombo BM, Sprecacenere B, Caiti M, Massa G, Picciotto A, Ceppa P, Puppo F. Autoimmune intrahepatic cholangiopathy associated with antiphospholipid antibody syndrome. Eur. J. Gastroenterol. Hepatol. 19: 910-912 (2007).

• Musiek ES, Gao L, Milne GL, Han W, Everhart MB, Wang D, Backlund MG, Dubois RN, Zanoni G, Vidari G, Blackwell TS, Morrow JD. Cyclopentenone isoprostanes inhibit the inflammatory response in macrophages. J. Biol. Chem. 280: 35562-35570 (2005). *

• Mutimer DJ, Fussey SP, Yeaman SJ, Kelly PJ, James OF, Bassendine MF. Frequency of IgG and IgM autoantibodies to four specific M2 mitochondrial autoantigens in primary biliary cirrhosis. Hepatology 10: 403-407 (1989).

• Muting D, Fischer R, Kalk JF, Kruck P. Chronic destructive non-suppurating cholangitis. Fortschr. Med. 100: 1179-1187 (1982).

• Mzali S, Johanet C, Chretien P, Abuaf N. Antinuclear antibodies in primary biliary cirrhosis. Gastroenterol. Clin. Biol. 13: 690-695 (1989).

• Nachbar F, Korting HC, Hoffmann RM, Kollmann M, Meurer M. Unusual coexistence of systemic lupus erythematosus and primary biliary cirrhosis. Dermatology 188: 313-317 (1994).

• Nagai S, Manns M, Meyer zum Buschenfelde KH, Dienes HP. Detection of mitochondrial antibodies directed against the primary biliary cirrhosis (M2) antigen by an enzyme-linked immunosorbent assay (ELISA). J. Immunol. Methods 60: 77-88 (1983).

• Nagothu KK, Bhatt R, Kaushal GP, Portilla D. Fibrate prevents cisplatin-induced proximal tubule cell death. Kidney Int. 68: 2680-2693 (2005). *

• Nagy G, Geher P, Koncz A, Perl A. Signal transduction abnormalities in systemic lupus erythematosus. Orv. Hetil. 146: 1625-1630 (2005).

• Nagy G, Koncz A, Perl A. T- and B-cell abnormalities in systemic lupus erythematosus. Crit. Rev. Immunol. 25: 123-140 (2005).

• Nagy G, Perl A. The role of nitric oxide in abnormal T cell signal transduction in systemic lupus erythematosus. Clin. Immunol. 118: 145-151 (2006). *

• Nakajima A, Kurihara H, Yagita H, Okumura K, Nakano H. Mitochondrial extrusion through the cytoplasmic vacuoles during cell death. J. Biol. Chem. 283: 24128-24135 (2008). *

• Nakajima M, Shimizu H, Miyazaki A, Watanabe S, Kitami N, Sato N. Detection of IgA, IgM, and IgG subclasses of anti-M2 antibody by immunoblotting in autoimmune cholangitis: is autoimmune cholangitis an early stage of primary biliary cirrhosis? J. Gastroenterol. 34: 607-612 (1999). *

• Nakamura M. The significance of anti-nuclear envelope (gp210) antibody in primary biliary cirrhosis. Nihon Rinsho Meneki Gakkai Kaishi 28: 117-122 (2005). *

• Nakamura M, Ishibashi H, Matsui M, Shimoda S, Hayashida K, Koike K, Niho Y. Peripheral B lymphocyte repertoire to mitochondrial antigen in primary biliary cirrhosis - positive correlation between the disease activity and the frequency of circulating B lymphocytes specific for pyruvate dehydrogenase complex. Autoimmunity 21: 253-262 (1995).

• Nakamura RM, Chisari FV, Edgington TS. Laboratory tests for diagnosis of autoimmune diseases. Prog. Clin. Pathol. 6: 177-203 (1975).

• Nakamura T, Higashi SI, Tomoda K, Tsukano M, Sugi K. Primary biliary cirrhosis (PBC)-CREST overlap syndrome with coexistence of Sjogren's syndrome and thyroid dysfunction. Clin. Rheumatol. 26: 596-600 (2007). *

• Nakamura T, Kawagoe Y, Ueda Y, Koide H. Antineutrophil cytoplasmic autoantibody-associated rapidly progressive glomerulonephritis in a patient with primary biliary cirrhosis. Am. J. Med. Sci. 328: 176-179 (2004).

• Nakamuta M, Enjoji M, Kotoh K, Shimohashi N, Tanabe Y. Long-term fibrate treatment for PBC. J. Gastroenterol. 40: 546-547 (2005). *

• Nakano T, Ishibashi H, Matsui M, Yasutake T, Ishii Y, Dohmen K, Nagasawa K, Niho Y. A case of primary biliary cirrhosis with negative anti-mitochondrial antibody anteceded by rheumatoid arthritis. Fukuoka Igaku Zasshi 83: 144-148 (1992).

• Nakanuma Y, Harada K, Kaji K, Terasaki S, Tsuneyama K, Moteki S, Van de Water J, Leung PS, Gershwin ME. Clinicopathological study of primary biliary cirrhosis negative for antimitochondrial antibodies. Liver 17: 281-287 (1997).

• Nakanuma Y, Kato Y, Unoura M, Kobayashi K, Yamamoto M. An autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver. Acta Pathol. Jpn. 38: 249-257 (1988).

• Nakanuma Y, Kouda W, Harada K, Hiramatsu K. Hepatic sarcoidosis with vanishing bile duct syndrome, cirrhosis, and portal phlebosclerosis. Report of an autopsy case. J. Clin. Gastroenterol. 32: 181-184 (2001).

• Nakanuma Y, Tsuneyama K, Kono N, Hoso M, Van de Water J, Gershwin ME. Biliary epithelial expression of pyruvate dehydrogenase complex in primary biliary cirrhosis: an immunohistochemical and immunoelectron microscopic study. Hum. Pathol. 26: 92-98 (1995).

• Nakanuma Y, Yasoshima M, Morita T, Gershwin ME. Abnormal expression of pyruvate dehydrogenase on bile ducts opens new fields in the pathogenesis of primary biliary cirrhosis. Nippon Rinsho 53: 1287-1296 (1995).

• Nalbandian G, Van de Water J, Gish R, Manns M, Coppel RL, Rudich SM, Prindiville T, Gershwin ME. Is there a serological difference between men and women with primary biliary cirrhosis? Am. J. Gastroenterol. 94: 2482-2486 (1999). *

• Nancey S, Blanvillain E, Parmentier B, Flourie B, Bayet C, Bienvenu J, Fabien N. Infliximab treatment does not induce organ-specific or nonorgan-specific autoantibodies other than antinuclear and anti-double-stranded DNA autoantibodies in Crohn's disease. Inflamm. Bowel Dis. 11: 986-991 (2005). *

• Nardi N, Brito-Zeron P, Ramos-Casals M, Aguilo S, Cervera R, Ingelmo M, Font J. Circulating auto-antibodies against nuclear and non-nuclear antigens in primary Sjogren's syndrome. Prevalence and clinical significance in 335 patients. Clin. Rheumatol. 25: 341-346 (2006). *

• Natarajan SK, Basivireddy J, Ramachandran A, Thomas S, Ramamoorthy P, Pulimood AB, Jacob M, Balasubramanian KA. Renal damage in experimentally-induced cirrhosis in rats: role of oxygen free radicals. Hepatology 43: 1248-1256 (2006). *

• Natarajan SK, Thangaraj KR, Eapen CE, Ramachandran A, Mukhopadhya A, Mathai M, Seshadri L, Peedikayil A, Ramakrishna B, Balasubramanian KA. Liver injury in acute fatty liver of pregnancy: possible link to placental mitochondrial dysfunction and oxidative stress. Hepatology 51: 191-200 (2010). *

• Naugler KM, Baer KA, Ropeleski MJ. Interleukin-11 antagonizes Fas ligand-mediated apoptosis in IEC-18 intestinal epithelial crypt cells: role of MEK1, 2 and Akt-dependent signaling. Am. J. Physiol. Gastrointest. Liver Physiol. 294: G728-G737 (2008). *

• Naviaux RK, Le TP, Bedelbaeva K, Leferovich J, Gourevitch D, Sachadyn P, Zhang XM, Clark L, Heber-Katz E. Retained features of embryonic metabolism in the adult MRL mouse. Mol. Genet. Metab. 96: 133-144 (2009). *

• Naziroglu M, Cihangir Uguz A, Kocak A, Bal R. Acetaminophen at different doses protects brain microsomal Ca(2+)-ATPase and the antioxidant redox system in rats. J. Membr. Biol. Oct 17 [Epub ahead of print] (2009). *

• Nedergaard J, Golozoubova V, Matthias A, Asadi A, Jacobsson A, Cannon B. UCP1: the only protein able to mediate adaptive non-shivering thermogenesis and metabolic inefficiency. Biochim. Biophys. Acta 1504: 82-106 (2001). *

• Nemec KN, Khaled AR. Therapeutic modulation of apoptosis: targeting the BCL-2 family at the interface of the mitochondrial membrane. Yonsei Med. J. 49: 689-697 (2008). *

• Neuberger J. Liver transplantation for autoimmune liver diseases. Falk Symposium 142: GASTROENTEROLOGY WEEK FREIBURG 2004 (Part I) AUTOIMMUNE LIVER DISEASE, Freiburg (Germany), October 12 - 13, 2004, pp. 82-83 (2004). *

• Neuberger J. Current problems in primary biliary cirrhosis. Z. Gastroenterol. 31 Suppl. 2: 90-93 (1993).

• Neuberger J. Recurrence of autoimmune liver diseases after liver transplantation. Falk Symposium 135. Immmunological diseases of liver and gut. Prague (Czech Republic). p. 47-48. September 12 - 13 (2003). *

• Neuberger J. Liver transplantation for cholestatic liver disease. Curr. Treat. Options Gastroenterol. 6: 113-121 (2003).

• Neuberger J. Recurrent primary biliary cirrhosis. Best Pract. Res. Clin. Gastroenterol. 14: 669-680 (2000). *

• Neuberger J, Bradwell AR. Anti-mitochondrial antibodies in primary biliary cirrhosis. J. Hepatol. 37: 712-716 (2002). *

• Neurath MF. Immunosuppressive drugs: clinical use and mechanism of action. Falk Symposium 146: GUT-LIVER INTERACTIONS: BASIC AND CLINICAL CONCEPTS, Innsbruck (Austria), March 11-12, 2005, p. 31 (2005). *

• Neurath MF. Apoptosis as a target. Falk Symposium 140. Inflammatory Bowel Disease: Translation from Basic Research to Clinical Practice. Dubrovnik (Croatia). p. 62. May 7-8 (2004). *

• Neustadt J, Pieczenik SR. Medication-induced mitochondrial damage and disease. Mol. Nutr. Food Res. 52: 780-788 (2008). *

• Newton J, Poyner E, Jones DE. Serum anti-mitochondrial antibodies and fatigue in primary biliary cirrhosis. Liver Int. 29: 1285-1286 (2009). *

• Newton JL, Jones DE, Metcalf JV, Park JB, Burt AD, Bassendine MF, James OF. Presentation and mortality of primary biliary cirrhosis in older patients. Age Ageing 29: 305-309 (2000). *

• Nezu S, Tanaka A, Yasui H, Imamura M, Nakajima H, Ishida H, Takahashi SI. Presence of antimitochondrial autoantibodies in patients with autoimmune hepatitis. J. Gastroenterol. Hepatol. 21: 1448-1454 (2006). *

• Ng Y, Barhoumi R, Tjalkens RB, Fan YY, Kolar S, Wang N, Lupton JR, Chapkin RS. The role of docosahexaenoic acid in mediating mitochondrial membrane lipid oxidation and apoptosis in colonocytes. Carcinogenesis 26: 1914-1921 (2005). *

• Nickowitz RE, Wozniak RW, Schaffner F, Worman HJ. Autoantibodies against integral membrane proteins of the nuclear envelope in patients with primary biliary cirrhosis. Gastroenterology 106: 193-199 (1994).

• Nijhawan PK, Therneau TM, Dickson ER, Boynton J, Lindor KD. Incidence of cancer in primary biliary cirrhosis: the Mayo experience. Hepatology 29: 1396-1398 (1999). *

• Ninova-Bradistilova D, Naumov N, Krustev Z, Ognianov M, Maleev A. Significance of high titers of various autoantibodies in the diagnosis and monitoring of chronic liver diseases. Vutr. Boles. 22: 46-52 (1983).

• Nishiki M, Murakami Y, Koshimura K, Sohmiya M, Tanaka J, Yabe S, Kobayashi I, Kato Y. A case of autoimmune hypophysitis associated with asymptomatic primary biliary cirrhosis. Endocr. J. 45: 697-700 (1998).

• Nishimori I, Akisawa N, Miyaji E, Kohsaki T, Iwasaki S, Onishi S. Serum antibody to carbonic anhydrase II in patients with chronic viral hepatitis: a review of its prevalence in liver diseases. Hepatol. Res. 30: 210-213 (2004).

• Nishio A, Bass NM, Luketic VA, Coppel RL, Gershwin ME. Primary biliary cirrhosis: from induction to destruction. Semin. Gastrointest. Dis. 12: 89-102 (2001).

• Nishio A, Coppel R, Ishibashi H, Gershwin ME. The pyruvate dehydrogenase complex as a target autoantigen in primary biliary cirrhosis. Baillieres Best Pract. Res. Clin. Gastroenterol. 14: 535-547 (2000).

• Nishio A, Keeffe EB, Gershwin ME. Immunopathogenesis of primary biliary cirrhosis. Semin. Liver Dis. 22: 291-302 (2002). *

• Nishio A, Keeffe EB, Gershwin ME. Primary biliary cirrhosis: lessons learned from an organ-specific disease. Clin. Exp. Med. 1: 165-178 (2001). *

• Nishio A, Keeffe EB, Ishibashi H, Gershwin EM. Diagnosis and treatment of primary biliary cirrhosis. Med. Sci. Monit. 6: 181-193 (2000). *

• Nishio A, Neuberger J, Gershwin ME. Management of patients with primary biliary cirrhosis: a practical guide. BioDrugs 12: 159-173 (1999).

• Nishio A, Van de Water J, Leung PS, Joplin R, Neuberger JM, Lake J, Bjorkland A, Totterman TH, Peters M, Worman HJ, Ansari AA, Coppel RL, Gershwin ME. Comparative studies of antimitochondrial autoantibodies in sera and bile in primary biliary cirrhosis. Hepatology 25: 1085-1089 (1997). *

• No authors listed. In this issue. Eur. J. Immunol. 38: 895-896 (2008).

• Nocito A, Dahm F, Jochum W, Jang JH, Georgiev P, Bader M, Renner EL, Clavien PA. Serotonin mediates oxidative stress and mitochondrial toxicity in a murine model of nonalcoholic steatohepatitis. Gastroenterology 133: 608-618 (2007). *

• Norman GL, Bialek A, Encabo S, Butkiewicz B, Wiechowska-Kozlowska A, Brzosko M, Shums Z, Milkiewicz P. Is prevalence of PBC underestimated in patients with systemic sclerosis? Dig. Liver Dis. 41: 762-764 (2009).

• Nunn AV, Bell J, Barter P. The integration of lipid-sensing and anti-inflammatory effects: how the PPARs play a role in metabolic balance. Nucl. Recept. 5: 1 (2007). *

• O'Brien C, Joshi S, Feld JJ, Guindi M, Dienes HP, Heathcote EJ. Long-term follow-up of antimitochondrial antibody-positive autoimmune hepatitis. Hepatology 48: 550-556 (2008). *

• O'Donohue J, McFarlane B, Bomford A, Yates M, Williams R. Antibodies to atypical mycobacteria in primary biliary cirrhosis. J. Hepatol. 21: 887-999 (1994).

• O'Donohue J, Williams R. Antimitochondrial antibody and primary biliary cirrhosis: can there be one without the other? J. Hepatol. 25: 574-577 (1996). *

• O'Donohue J, Wong T, Portmann B, Williams R. Immunohistochemical differences in the portal tract and acinar infiltrates between primary biliary cirrhosis and autoimmune cholangitis. Eur. J. Gastroenterol. Hepatol. 14: 1143-1150 (2002).

• Obukhova LA, Skulachev VP, Kolosova NG. Mitochondria-targeted antioxidant SkQ1 inhibits age-dependent involution of the thymus in normal and senescence-prone rats. Aging (Albany NY) 1: 389-401 (2009).

• Odin JA, Huebert RC, Casciola-Rosen L, LaRusso NF, Rosen A. Bcl-2-dependent oxidation of pyruvate dehydrogenase-E2, a primary biliary cirrhosis autoantigen, during apoptosis. J. Clin. Invest. 108: 223-232 (2001). *

• Oertelt S, Kenny TP, Selmi C, Invernizzi P, Podda M, Gershwin ME. SNP analysis of genes implicated in T cell proliferation in primary biliary cirrhosis. Clin. Dev. Immunol. 12: 259-263 (2005). *

• Oertelt S, Lian ZX, Cheng CM, Chuang YH, Padgett KA, He XS, Ridgway WM, Ansari AA, Coppel RL, Li MO, Flavell RA, Kronenberg M, Mackay IR, Gershwin ME. Anti-mitochondrial antibodies and primary biliary cirrhosis in TGF-beta receptor II dominant-negative mice. J. Immunol. 177: 1655-1660 (2006). *

• Oertelt S, Ridgway WM, Ansari AA, Coppel RL, Gershwin ME. Murine models of primary biliary cirrhosis: comparisons and contrasts. Hepatol. Res. 37 Suppl. 3: S365-S369 (2007). *

• Oertelt S, Rieger R, Selmi C, Invernizzi P, Ansari AA, Coppel RL, Podda M, Leung PS, Gershwin ME. A sensitive bead assay for antimitochondrial antibodies: chipping away at AMA-negative primary biliary cirrhosis. Hepatology 45: 659-665 (2007). *

• Ohashi K, Yokoyama T, Yamato M, Kuge H, Kanehiro H, Tsutsumi M, Amanuma T, Iwata H, Yang J, Okano T, Nakajima Y. Engineering functional two- and three-dimensional liver systems in vivo using hepatic tissue sheets. Nat. Med. 13: 880-885 (2007). *

• Ohba K, Omagari K, Kinoshita H, Soda H, Masuda J, Hazama H, Tagawa M, Hata T, Nakamura H, Murata I, Kohno S. Primary biliary cirrhosis among atomic bomb survivors in Nagasaki, Japan. J. Clin. Epidemiol. 54: 845-850 (2001).

• Ohba K, Omagari K, Murase K, Hazama H, Masuda J, Kinoshita H, Isomoto H, Mizuta Y, Miyazaki M, Murata I, Kohno S. A possible mouse model for spontaneous cholangitis: serological and histological characteristics of MRL/lpr mice. Pathology 34: 250-256 (2002). *

• Ohge H, Takesue Y, Yokoyama T, Hiyama E, Murakami Y, Imamura Y, Shimamoto F, Matsuura Y. Progression of primary biliary cirrhosis after proctocolectomy for ulcerative colitis. J. Gastroenterol. 35: 870-872 (2000). *

• Ohira H, Takiguchi J, Rai T, Shishido S, Sato Y. Two cases of primary biliary cirrhosis complicated with syringomyelia. Am. J. Gastroenterol. 98: 224-226 (2003). *

• Ohno N, Ota Y, Hatakeyama S, Yanagimoto S, Morisawa Y, Tsukada K, Koike K, Kimura S. A patient with E. coli-induced pyelonephritis and sepsis who transiently exhibited symptoms associated with primary biliary cirrhosis. Intern. Med. 42: 1144-1148 (2003). *

• Ohtsuka T. Relation between elevated high-sensitivity C-reactive protein and anti-mitochondria antibody in patients with systemic sclerosis. J. Dermatol. 35: 70-75 (2008). *

• Okada C, Akbar SM, Horiike N, Onji M. Early development of primary biliary cirrhosis in female C57BL/6 mice because of poly I:C administration. Liver Int. 25: 595-603 (2005). *

• Okazaki K, Uchida K, Ohana M, Nakase H, Uose S, Inai M, Matsushima Y, Katamura K, Ohmori K, Chiba T. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology 118: 573-581 (2000). *

• Okiyama W, Tanaka N, Nakajima T, Tanaka E, Kiyosawa K, Gonzalez FJ, Aoyama T. Polyenephosphatidylcholine prevents alcoholic liver disease in PPARalpha-null mice through attenuation of increases in oxidative stress. J. Hepatol. 50: 1236-1246 (2009).

• Okolicsanyi L, Fabris L. Diagnostic approach to primary sclerosing cholangitis: open questions. Ital. J. Gastroenterol. 23: 436-441 (1991).

• Olafsson S, Gudjonsson H, Selmi C, Amano K, Invernizzi P, Podda M, Gershwin ME. Antimitochondrial antibodies and reactivity to N. aromaticivorans proteins in Icelandic patients with primary biliary cirrhosis and their relatives. Am. J. Gastroenterol. 99: 2143-2146 (2004). *

• Oliveira MR, Oliveira MW, Behr GA, Moreira JC. Vitamin A supplementation at clinical doses induces a dysfunction in the redox and bioenergetics states, but did change neither caspases activities nor TNF-alpha levels in the frontal cortex of adult Wistar rats. J. Psychiatr. Res. 43: 754-762 (2009).

• Oliver-Minarro D, Sanchez-Ramon S, Rodriguez-Mahou M, Alvarez S, Fernandez-Cruz E. Isolated type 5 antimitochondrial autoantibodies are associated with a history of thrombocytopenia and fetal loss. Fertil. Steril. 87: 976.e17-e18 (2007).

• Omagari K. Autoimmune cholangitis: is it an antimitochondrial antibody negative primary biliary cirrhosis? Acta Medica Nagasakiensia 44: 11-14 (1999). *

• Omagari K, Hazama H, Kohno S. Enzyme inhibition assay for pyruvate dehydrogenase complex: clinical utility for the diagnosis of primary biliary cirrhosis. World J. Gastroenterol. 11: 6735-6739 (2005). *

• Omagari K, Ikuno N, Matsuo I, Shirono K, Hara K, Feeney SJ, Whittingham S, Mackay IR. Autoimmune cholangitis syndrome with a bias towards primary biliary cirrhosis. Pathology 28: 255-258 (1996).

• Omagari K, Kadokawa Y, Nakamura M, Akazawa S, Ohba K, Ohnita K, Mizuta Y, Daikoku M, Yatsuhashi H, Ishibashi H, Kohno S. IgA class antibodies to 2-oxo-acid dehydrogenase complex are not predictive markers of histopathological progression in primary biliary cirrhosis. Autoimmunity 39: 107-112 (2006).

• Omagari K, Masuda J, Kato Y, Nakata K, Kanematsu T, Kusumoto Y, Mori I, Furukawa R, Tanioka H, Tajima H, Koga M, Yano M, Kohno S. Re-analysis of clinical features of 89 patients with autoimmune hepatitis using the revised scoring system proposed by the International Autoimmune Hepatitis Group. Intern. Med. 39: 1008-1012 (2000). *

• Omagari K, Rowley MJ, Jois JA, Feeney SJ, Komatsu K, Maeda T, Onishi S, Yamazaki K, Suzuki K, Galperin C, Mackay IR. Immunoreactivity of antimitochondrial autoantibodies in Japanese patients with primary biliary cirrhosis. J. Gastroenterol. 31: 61-68 (1996).

• Omagari K, Rowley MJ, Whittingham S, Jois JA, Byron SL, Mackay IR. Autoantibodies to M2 mitochondrial autoantigens in normal human sera by immunofluorescence and novel assays. J. Gastroenterol. Hepatol. 11: 610-616 (1996).

• Onal IK, Ersoy O, Aydinli M, Yonem O, Harmanci O, Sokmensuer C, Bayraktar Y. Hepatic granuloma in Turkish adults: a report of 13 cases. Eur. J. Intern. Med. 19: 527-530 (2008).

• Ono M, Niinobu T, Yamanaka Y, Yasuda O, Rakugi H, Ikegami H, Miki T, Ogihara T. Primary biliary cirrhosis with polymyositis successfully treated with prednisolone and ursodeoxycholic acid. Nippon Ronen Igakkai Zasshi 33: 779-782 (1996).

• Onozuka Y, Shibata M, Yonezawa H, Terauti K, Miyachi K, Ueno Y. Clinical significance of anti-centromere antibody and anti-CENP-B antibody in sera of patients with primary biliary cirrhosis. Rinsho Byori 44: 877-882 (1996).

• Oo YH, Neuberger J. Options for treatment of primary biliary cirrhosis. Drugs 64: 2261-2271 (2004).

• Oruc N, Lamb J, Whitcomb DJ, Sass DA. The ACE gene I/D polymorphism does not affect the susceptibility to or prognosis of PBC. Turk. J. Gastroenterol. 19: 250-253 (2008). *

• Otani K, Korenaga M, Beard MR, Li K, Qian T, Showalter LA, Singh AK, Wang T, Weinman SA. Hepatitis C virus core protein, cytochrome P450 2E1, and alcohol produce combined mitochondrial injury and cytotoxicity in hepatoma cells. Gastroenterology 128: 96-107 (2005). *

• Padgett KA, Selmi C, Kenny TP, Leung PS, Balkwill DL, Ansari AA, Coppel RL, Gershwin ME. Phylogenetic and immunological definition of four lipoylated proteins from Novosphingobium aromaticivorans, implications for primary biliary cirrhosis. J. Autoimmun. 24: 209-219 (2005).

• Pajak B, Gajkowska B, Orzechowski A. Sodium butyrate sensitizes human colon adenocarcinoma COLO 205 cells to both intrinsic and TNF-alpha-dependent extrinsic apoptosis. Apoptosis 14: 203-217 (2009). *

• Palmer JM, Bassendine MF, James OF, Yeaman SJ. Human pyruvate dehydrogenase complex as an autoantigen in primary biliary cirrhosis. Clin. Sci. (Lond.) 85: 289-293 (1993).

• Palmer JM, Yeaman SJ, Bassendine MF, James OF. M4 and M9 autoantigens in primary biliary cirrhosis - a negative study. J. Hepatol. 18: 251-254 (1993).

• Panasiuk A, Prokopowicz D, Zak J. Peripheral blood T, B lymphocytes and NK cells in primary biliary cirrhosis. Rocz. Akad. Med. Bialymst. 46: 231-239 (2001).

• Pares A. Natural history of primary biliary cirrhosis. Gastroenterol. Hepatol. 31: 500-507 (2008).

• Parveen S, Morshed SA, Nishioka M. High prevalence of antibodies to recombinant CENP-B in primary biliary cirrhosis: nuclear immunofluorescence patterns and ELISA reactivities. J. Gastroenterol. Hepatol. 10: 438-445 (1995).

• Pascual I, Larrayoz IM, Campos MM, Rodriguez IR. Methionine sulfoxide reductase b2 is highly expressed in the retina and protects retinal pigmented epithelium cells from oxidative damage. Exp. Eye Res. Dec 18 [Epub ahead of print] (2009). *

• Patel D, Egner W, Gleeson D, Wild G, Ward A. Detection of serum M2 anti-mitochondrial antibodies by enzyme-linked immunosorbent assay is potentially less specific than by immunofluorescence. Ann. Clin. Biochem. 39: 304-307 (2002).

• Pathan N, Marusawa H, Krajewska M, Matsuzawa S, Kim H, Okada K, Torii S, Kitada S, Krajewski S, Welsh K, Pio F, Godzik A, Reed JC. TUCAN, an antiapoptotic caspase-associated recruitment domain family protein overexpressed in cancer. J. Biol. Chem. 276: 32220-32229 (2001). *

• Patriarca F, Skert C, Sperotto A, Zaja F, Falleti E, Mestroni R, Kikic F, Calistri E, Fili C, Geromin A, Cerno M, Fanin R. The development of autoantibodies after allogeneic stem cell transplantation is related with chronic graft-vs-host disease and immune recovery. Exp. Hematol. 34: 389-396 (2006). *

• Paunel-Gorgulu A, Zornig M, Logters T, Altrichter J, Rabenhorst U, Cinatl J, Windolf J, Scholz M. Mcl-1-mediated impairment of the intrinsic apoptosis pathway in circulating neutrophils from critically ill patients can be overcome by Fas stimulation. J. Immunol. 183: 6198-6206 (2009).

• Pavic S, Simonovic J, Boricic I, Svirtlih N. Autoantibodies characteristic for autoimmune hepatitis found in chronic hepatitis C. Srp. Arh. Celok. Lek. 131: 437-442 (2003).

• Pedreira JD, Guardia J, Benet JM, Martin C, Esteban R, Martinez-Vazquez JM, Bacardi R. Diagnostic value of anti-mitochondria antibodies. Nouv. Presse. Med. 6: 544-546 (1977).

• Pelissier MA, Marteau P, Pochart P. Antioxidant effects of metronidazole in colonic tissue. Dig. Dis. Sci. 52: 40-44 (2007). *

• Penna F, Reffo P, Muzio G, Canuto RA, Baccino FM, Bonelli G, Costelli P. Mechanisms of clofibrate-induced apoptosis in Yoshida AH-130 hepatoma cells. Biochem. Pharmacol. 77: 169-176 (2009). *

• Penner E, Goldenberg H, Meryn S, Gordon J. A dot-immunobinding assay for antimitochondrial antibodies. Hepatology 6: 381-384 (1986).

• Pepe S, Leong JY, Merwe JV, Marasco SF, Hadj A, Lymbury R, Perkins A, Rosenfeldt FL. Targeting oxidative stress in surgery: effects of ageing and therapy. Exp. Gerontol. 43: 653-657 (2008).

• Perez Garrigues H, Carmona E, Morera C, Sanchez-Cuenca JM. Circulating auto-antibodies in Meniere's disease. Ann. Otolaryngol. Chir. Cervicofac. 112: 225-228 (1995).

• Perez MJ, Castano B, Jimenez S, Serrano MA, Gonzalez-Buitrago JM, Marin JJ. Role of vitamin C transporters and biliverdin reductase in the dual pro-oxidant and anti-oxidant effect of biliary compounds on the placenta-fetal unit in cholestasis during pregnancy. Toxicol. Appl. Pharmacol. 232: 327-336 (2008). *

• Perez MJ, Macias RI, Marin JJ. Maternal cholestasis induces placental oxidative stress and apoptosis. Protective effect of ursodeoxycholic acid. Placenta 27: 34-41 (2006).

• Perl A. Pathogenic mechanisms in systemic lupus erythematosus. Autoimmunity Dec 17 [Epub ahead of print] (2009).

• Perros P, Palmer JM, Yeaman SJ, Kendall-Taylor P. Anti-mitochondrial antibodies in patients with Graves' disease may not signify primary biliary cirrhosis. Postgrad. Med. J. 70: 17-18 (1994). *

• Pessayre D, Berson A, Fromenty B, Mansouri A. Mitochondria in steatohepatitis. Semin. Liver Dis. 21: 57-69 (2001).

• Pessayre D, Fromenty B, Mansouri A. Mitochondrial injury in steatohepatitis. Eur. J. Gastroenterol. Hepatol. 16: 1095-1105 (2004).

• Peters MG, Di Bisceglie AM, Kowdley KV, Flye NL, Luketic VA, Munoz SJ, Garcia-Tsao G, Boyer TD, Lake JR, Bonacini M, Combes B; PUMPS Group. Differences between Caucasian, African American, and Hispanic patients with primary biliary cirrhosis in the United States. Hepatology 46: 769-775 (2007). *

• Pieczenik SR, Neustadt J. Mitochondrial dysfunction and molecular pathways of disease. Exp. Mol. Pathol. 83: 84-92 (2007). *

• Pinckard RN, Weir DM. Antibodies against the mitochondrial fraction of liver after toxic liver damage in rats. Clin. Exp. Immunol. 1: 33-43 (1966). *

• Pla X, Vergara M, Gil M, Dalmau B, Cistero B, Bella RM, Real J. Incidence, prevalence and clinical course of primary biliary cirrhosis in a Spanish community. Eur. J. Gastroenterol. Hepatol. 19: 859-864 (2007).

• Plodziszewska M, Slodkowska J, Orlowski T, Pawlicka L, Rowinska-Zakrzewska E. Primary biliary cirrhosis with sarcoid-like infiltrations in the lung. Pneumonol. Alergol. Pol. 66: 330-336 (1998).

• Polenakovic M, Cukaranovic R, Savic V, Jovanovic I, Stefanovic V. Immunological studies in Balkan Endemic Nephropathy. Prilozi. 28: 13-22 (2007).

• Pollak C, Minar E, Dragosics B, Marosi L. Primary biliary cirrhosis and scleroderma: long-term benign course of a complex autoimmune disease. Leber. Magen. Darm. 15: 85-89 (1985).

• Polman CH, Kappos L, Dahlke F, Graf R, Beckmann K, Bogumil T, Pozzilli C, Thompson AJ. Interferon beta-1b treatment does not induce autoantibodies. Neurology 64: 996-1000 (2005).

• Pompeia C, Lopes LR, Miyasaka CK, Procopio J, Sannomiya P, Curi R. Effect of fatty acids on leukocyte function. Braz. J. Med. Biol. Res. 33: 1255-1268 (2000). *

• Portincasa P, Grattagliano I, Palmieri VO, Palasciano G. Current pharmacological treatment of nonalcoholic fatty liver. Curr. Med. Chem. 13: 2889-2900 (2006).

• Portincasa P, Grattagliano I, Testini M, Caruso ML, Wang DQ, Moschetta A, Calamita G, Vacca M, Valentini AM, Renna G, Lissidini G, Palasciano G. Parallel intestinal and liver injury during early cholestasis in the rat: modulation by bile salts and antioxidants. Free Radic. Biol. Med. 42: 1381-1391 (2007).

• Portincasa P, Vacca M, Moschetta A, Petruzzelli M, Palasciano G, van-Erpecum KJ, van-Berge-Henegouwen GP. Primary sclerosing cholangitis: updates in diagnosis and therapy. World J. Gastroenterol. 11: 7-16 (2005). *

• Portmann BC. Primary biliary cirrhosis and sclerosing cholangitis. Current Diagnostic Pathology 8: 133-143 (2002). *

• Potter KN, Thomson RK, Hamblin A, Richards SD, Lindsay JG, Stevenson FK. Immunogenetic analysis reveals that epitope shifting occurs during B-cell affinity maturation in primary biliary cirrhosis. J. Mol. Biol. 306: 37-46 (2001). *

• Poupon R. Primary biliary cirrhosis. Schweiz. Med. Wochenschr. 121: 727-732 (1991).

• Poupon R. Primary biliary cirrhosis: a 2010 update. J. Hepatol. [In Press] (2010).

• Poupon R, Chazouilleres O, Poupon RE. Chronic cholestatic diseases. J. Hepatol. 32: S129-S140 (2000).

• Poupon R, Poupon RE. Retrovirus infection as a trigger for primary biliary cirrhosis? Lancet 363: 260-261 (2004). *

• Poupon RE, Balkau B, Eschwege E, Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study Group. N. Engl. J. Med. 324: 1548-1554 (1991).

• Prabhu RM, Medeiros LJ, Kumar D, Drachenberg CI, Papadimitriou JC, Appelman HD, Johnson LB, Laurin J, Heyman M, Abruzzo LV. Primary hepatic low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) associated with primary biliary cirrhosis. Mod. Pathol. 11: 404-410 (1998).

• Pratt DS. Cholestasis and cholestatic syndromes. Curr. Opin. Gastroenterol. 21: 270-274 (2005). *

• Premoli A, Morello E, Bo S, Durazzo M. Diagnostic and therapeutic questions in overlap syndromes of autoimmune hepatitis. Minerva Gastroenterol. Dietol. 53: 79-82 (2007).

• PreuB B, Berg C, Altenberend F, Gregor M, Stevanovic S, Klein R. Demonstration of autoantibodies to recombinant human sulphite oxidase in patients with chronic liver disorders and analysis of their clinical relevance. Clin. Exp. Immunol. 150: 312-321 (2007). *

• Prignano F, Betts CM, Lotti T. Vogt-Koyanagi-Harada disease and vitiligo: where does the illness begin? J. Electron Microsc. (Tokyo) 57: 25-31 (2008).

• Primo J, Miralles A, Ferrando J, Albert A, Julve R, Gomez A, Garcia L. Atypical primary biliary cirrhosis or autoimmune cholangitis? Gastroenterol. Hepatol. 22: 505-509 (1999).

• Prince M, Christensen E, Gluud C. Glucocorticosteroids for primary biliary cirrhosis. Cochrane Database Syst. Rev. 2: CD003778 (2005). *

• Prince MI, Jones DE. Primary biliary cirrhosis: new perspectives in diagnosis and treatment. Postgrad. Med. J. 76: 199-206 (2000). *

• Provenzano G, Diquattro O, Craxi A, Almasio P, Pinzello G, Marino L, Fiorentino G, Rinaldi F, Pagliaro L. Immunoblotting as a confirmatory test for antimitochondrial antibodies in primary biliary cirrhosis. Gut 34: 544-548 (1993). *

• Pua HH, Guo J, Komatsu M, He YW. Autophagy is essential for mitochondrial clearance in mature T lymphocytes. J. Immunol. 182: 4046-4055 (2009).

• Puri AS, Kumar N, Gondal R, Lamba GS, Jain M. Primary biliary cirrhosis: an Indian experience. Indian J. Gastroenterol. 20: 28-29 (2001).

• Quaranta S, Shulman H, Ahmed A, Shoenfeld Y, Peter J, McDonald GB, Van de Water J, Coppel R, Ostlund C, Worman HJ, Rizzetto M, Tsuneyama K, Nakanuma Y, Ansari A, Locatelli F, Paganin S, Rosina F, Manns M, Gershwin ME. Autoantibodies in human chronic graft-versus-host disease after hematopoietic cell transplantation. Clin. Immunol. 91: 106-116 (1999). *

• Quinn J, Diamond AG, Palmer JM, Bassendine MF, James OF, Yeaman SJ. Lipoylated and unlipoylated domains of human PDC-E2 as autoantigens in primary biliary cirrhosis: significance of lipoate attachment. Hepatology 18: 1384-1391 (1993).

• Quintana A, Schwindling C, Wenning AS, Becherer U, Rettig J, Schwarz EC, Hoth M. T cell activation requires mitochondrial translocation to the immunological synapse. Proc. Natl. Acad. Sci. U.S.A. 104: 14418-14423 (2007). *

• Quintanilla RA, Jin YN, Fuenzalida K, Bronfman M, Johnson GV. Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease. J. Biol. Chem. 283: 25628-25637 (2008). *

• Rain F, Durieu I, Mackiewitz R, Levrat R. Dermatopolymyositis and primary biliary cirrhosis. A rare association. Presse. Med. 25: 581-582 (1996).

• Ramakrishna B, Eapen CE, Kang G, Kurian G, Chandy GM. Familial intrahepatic cholestatic cirrhosis with positive antimitochondrial antibody: familial primary biliary cirrhosis. J. Clin. Gastroenterol. 30: 255-258 (2000).

• Ramos-Casals M, Pares A, Jara LJ, Solans R, Vinas O, Vazquez P, Sanchez-Tapias JM, Rodes J, Font J; the HISPAMEC Study Group. Antimitochondrial antibodies in patients with chronic hepatitis C virus infection: description of 18 cases and review of the literature. J. Viral Hepat. 12: 648-654 (2005).

• Ramos-Casals M, Sanchez-Tapias JM, Pares A, Forns X, Brito-Zeron P, Nardi N, Vazquez P, Velez D, Arias I, Bove A, Plaza J, Rodes J, Font J. Characterization and differentiation of autoimmune versus viral liver involvement in patients with Sjogren's syndrome. J. Rheumatol. 33: 1593-1599 (2006).

• Randle HW, Millns JL, Schroeter AL, Winkelmann RK. Cutaneous immunofluorescence in primary biliary cirrhosis. JAMA 246: 1679-1681 (1981).

• Randy LH, Guoying B. Agonism of peroxisome proliferator receptor-gamma may have therapeutic potential for neuroinflammation and Parkinson's disease. Curr. Neuropharmacol. 5: 35-46 (2007).

• Rangwala SM, Li X, Lindsley L, Wang X, Shaughnessy S, Daniels TG, Szustakowski J, Nirmala NR, Wu Z, Stevenson SC. Estrogen-related receptor alpha is essential for the activation of genes of the antioxidant stress protection pathway and mitochondrial function. Biochem. Biophys. Res. Commun. 357: 231-236 (2007). *

• Rao NA, Saraswathy S, Wu GS, Katselis GS, Wawrousek EF, Bhat S. Elevated retina-specific expression of the small heat shock protein, alphaA-crystallin, is associated with photoreceptor protection in experimental uveitis. Invest. Ophthalmol. Vis. Sci. 49: 1161-1171 (2008). *

• Rautiainen H, Salomaa V, Niemela S, Karvonen AL, Nurmi H, Isoniemi H, Farkkila M. Prevalence and incidence of primary biliary cirrhosis are increasing in Finland. Scand. J. Gastroenterol. 42: 1347-1353 (2007). *

• Reggiani A, Dizioli P, Quattrocchi D, Quinzani M, Marelli A, Bodini P. Intrahepatic cholestasis: clinical and nosological classification. Critical survey of personal experience. Minerva Gastroenterol. Dietol. 43: 71-81 (1997).

• Reinehr R, Becker S, Keitel V, Eberle A, Grether-Beck S, Haussinger D. Bile salt-induced apoptosis involves NADPH oxidase isoform activation. Gastroenterology 129: 2009-2031 (2005). *

• Reisman Y, Gips CH, Lavelle SM. Primary biliary cirrhosis: an electronic diagnostic tool trial based on symptoms, (past) history and signs only, using the European database euricterus. The Euricterus PMG. Hepatogastroenterology 44: 1104-1109 (1997).

• Remacle JP, Pellissier JF, Chamlian A, Benkoel L, Aubert L, Monges H. Progressive ophthalmoplegia associated with asymptomatic primary biliary cirrhosis. Histologic, histochemical, cytochemical, and ultrastructural studies of muscle and liver biopsy specimens. Hum. Pathol. 11: S540-S548 (1980).

• Remmel T, Remmel H, Uibo R, Salupere V. Primary biliary cirrhosis in Estonia. With special reference to incidence, prevalence, clinical features, and outcome. Scand. J. Gastroenterol. 30: 367-371 (1995).

• Ren Y, Sun C, Sun Y, Tan H, Wu Y, Cui B, Wu Z. PPAR gamma protects cardiomyocytes against oxidative stress and apoptosis via Bcl-2 upregulation. Vascul. Pharmacol. 51: 169-174 (2009). *

• Reshetnyak VI. Concept on the pathogenesis and treatment of primary biliary cirrhosis. World J. Gastroenterol. 12: 7250-7262 (2006). *

• Reynoso-Paz S, Coppel RL, Nakanuma Y, Gershwin ME. Primary biliary cirrhosis. Connecting molecular biology to clinical medicine. Clin. Rev. Allergy Immunol. 18: 241-262 (2000).

• Reynoso-Paz S, Leung PS, Van De Water J, Tanaka A, Munoz S, Bass N, Lindor K, Donald PJ, Coppel RL, Ansari AA, Gershwin ME. Evidence for a locally driven mucosal response and the presence of mitochondrial antigens in saliva in primary biliary cirrhosis. Hepatology 31: 24-29 (2000). *

• Richards A, Rooney J, Prime S, Scully C. Primary biliary cirrhosis. Sole presentation with rampant dental caries. Oral Surg. Oral Med. Oral Pathol. 77: 16-18 (1994).

• Rieger R, Gershwin ME. The X and why of xenobiotics in primary biliary cirrhosis. J. Autoimmun. 28: 76-84 (2007). *

• Rieger R, Leung PS, Jeddeloh MR, Kurth MJ, Nantz MH, Lam KS, Barsky D, Ansari AA, Coppel RL, Mackay IR, Gershwin ME. Identification of 2-nonynoic acid, a cosmetic component, as a potential trigger of primary biliary cirrhosis. J. Autoimmun. 27: 7-16 (2006).

• Rieger R, Oertelt S, Selmi C, Invernizzi P, Podda M, Gershwin ME. Decreased serum leptin levels in primary biliary cirrhosis: a link between metabolism and autoimmunity? Ann. N. Y. Acad. Sci. 1051: 211-217 (2005). *

• Rifle G, Mousson C, Hillon P, Marceau M, Chalopin JM, Tanter Y. Primary biliary cirrhosis: therapeutic trial using plasma exchange and immunosuppression. Preliminary results. Ann. Med. Interne (Paris) 139 Suppl. 1: 32-34 (1988).

• Rigamonti C, Shand LM, Feudjo M, Bunn CC, Black CM, Denton CP, Burroughs AK. Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Gut 55: 388-394 (2006). *

• Rigopoulou EI, Bogdanos DP, Liaskos C, Koutsoumpas A, Baum H, Vergani D, Dalekos GN. Anti-mitochondrial antibody immunofluorescent titres correlate with the number and intensity of immunoblot-detected mitochondrial bands in patients with primary biliary cirrhosis. Clin. Chim. Acta 380: 118-121 (2007). *

• Rigopoulou EI, Davies ET, Bogdanos DP, Liaskos C, Mytilinaiou M, Koukoulis GK, Dalekos GN, Vergani D. Antimitochondrial antibodies of immunoglobulin G3 subclass are associated with a more severe disease course in primary biliary cirrhosis. Liver Int. 27: 1226-1231 (2007). *

• Rinaldi S, Landucci F, De Gaudio AR. Antioxidant therapy in critically septic patients. Curr. Drug Targets 10: 872-880 (2009).

• Rintahaka J, Wiik D, Kovanen PE, Alenius H, Matikainen S. Cytosolic antiviral RNA recognition pathway activates caspases 1 and 3. J. Immunol. 180: 1749-1757 (2008). *

• Risques RA, Rabinovitch PS, Brentnall TA. Cancer surveillance in inflammatory bowel disease: new molecular approaches. Curr. Opin. Gastroenterol. 22: 382-390 (2006).

• Ritzel U, Leonhardt U, Nather M, Schafer G, Armstrong VW, Ramadori G. Simvastatin in primary biliary cirrhosis: effects on serum lipids and distinct disease markers. J. Hepatol. 36: 454-458 (2002). *

• Robert K, Nehme J, Bourdon E, Pivert G, Friguet B, Delcayre C, Delabar JM, Janel N. Cystathionine beta synthase deficiency promotes oxidative stress, fibrosis, and steatosis in mice liver. Gastroenterology 128: 1405-1415 (2005). *

• Roberts KM, Rosen A, Casciola-Rosen LA. Methods for inducing apoptosis. Methods Mol. Med. 102: 115-128 (2004).

• Rocklin RE, Maxwell NR, Thistle L, Kaplan MM. In vitro anti-mitochondrial antibody synthesis in patients with primary biliary cirrhosis. Clin. Immunol. Immunopathol. 38: 22-31 (1986).

• Rodrigues CE, Borges CL, de Carvalho JF. Diffuse systemic sclerosis and autoimmune hepatitis: a unique association. Clin. Rheumatol. Feb 7 [Epub ahead of print] (2010). *

• Rodrigues CM, Fan G, Ma X, Kren BT, Steer CJ. A novel role for ursodeoxycholic acid in inhibiting apoptosis by modulating mitochondrial membrane perturbation. J. Clin. Invest. 101: 2790-2799 (1998). *

• Rodrigues CMP, Keene CD, Duan W-M, Sola S, Castro RE, Kren BT, Low WC, Steer CJ. Tauroursodeoxycholic acid, a bile acid with in vivo anti-apoptotic and neuroprotective properties. Falk Symposium 141. XVIII International Bile Acid Meeting: Bile Acid Biology and its Therapeutic Implications. Stockholm (Sweden). p. 64. June 18-19 (2004). *

• Rohrbach S. Effects of dietary polyunsaturated fatty acids on mitochondria. Curr. Pharm. Des. 15: 4103-4116 (2009).

• Rolo AP, Palmeira CM, Wallace KB. Mitochondrially mediated synergistic cell killing by bile acids. Biochim. Biophys. Acta 1637: 127-132 (2003). *

• Romero Gomez M, Wichmann I, Otero Fernandez MA, Suarez Garcia E, Grande L, Nunez J, Nevado Santos M, Castro Fernandez M. Anti-Sp100 and anti-Gp210 in the diagnosis of primary biliary cirrhosis in patients with autoimmune cholangitis. Gastroenterol. Hepatol. 22: 11-13 (1999).

• Romero-Gomez M, Wichmann I, Crespo J, Pares A, Rodrigo L, Alvarez A, Diago M, Pons-Romero F, Sanchez-Munoz D, Aguilar-Reina J, Andrade RJ, Salmeron J, Sanchez-Pobre P, Rebollo JM, Martin-Vivaldi R, Castellano-Megias V, Nunez-Roldan A, Bruguera M. Serum immunological profile in patients with chronic autoimmune cholestasis. Am. J. Gastroenterol. 99: 2150-2157 (2004). *

• Ronco MT, Frances DE, Ingaramo PI, Quiroga AD, Alvarez ML, Pisani GB, Revelli SS, Carnovale CE. Tumor necrosis factor alpha induced by Trypanosoma cruzi infection mediates inflammation and cell death in the liver of infected mice. Cytokine Nov 3 [Epub ahead of print] (2009).

• Rosenberg JL, Edlow D, Sneider R. Liver disease and vasculitis in a patient taking cromolyn. Arch. Intern. Med. 138: 989-991 (1978).

• Roth M, Black JL. An imbalance in C/EBPs and increased mitochondrial activity in asthmatic airway smooth muscle cells: novel targets in asthma therapy? Br. J. Pharmacol. 157: 334-341 (2009).

• Roudebush M, Slabe T, Sundaram V, Hoppel CL, Golubic M, Stacey DW. Neurofibromin colocalizes with mitochondria in cultured cells. Exp. Cell Res. 236: 161-172 (1997). *

• Routsias JG, Tzioufas AG. Sjogren's syndrome - study of autoantigens and autoantibodies. Clin. Rev. Allergy Immunol. 32: 238-251 (2007). *

• Rowley MJ, Maeda T, Mackay IR, Loveland BE, McMullen GL, Tribbick G, Bernard CC. Differing epitope selection of experimentally-induced and natural antibodies to a disease-specific autoantigen, the E2 subunit of pyruvate dehydrogenase complex (PDC-E2). Int. Immunol. 4: 1245-1253 (1992).

• Rowley MJ, McNeilage LJ, Armstrong JM, Mackay IR. Inhibitory autoantibody to a conformational epitope of the pyruvate dehydrogenase complex, the major autoantigen in primary biliary cirrhosis. Clin. Immunol. Immunopathol. 60: 356-370 (1991).

• Rubel LR, Rabin L, Seeff LB, Licht H, Cuccherini BA. Does primary biliary cirrhosis in men differ from primary biliary cirrhosis in women? Hepatology 4: 671-677 (1984).

• Rudnick DA, Perlmutter DH. Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease. Hepatology 42: 514-521 (2005). *

• Ruegg R, Matter L, Schopfer K, Grob PJ. Clinical significance of antimitochondrial antibodies. Schweiz. Med. Wochenschr. 115: 290-296 (1985).

• Ruhl R, Plum C, Elmazar MM, Nau H. Embryonic subcellular distribution of 13-cis- and all-trans-retinoic acid indicates differential cytosolic/nuclear localization. Toxicol. Sci. 63: 82-89 (2001). *

• Rust C, Bernt C, Vennegeerts T, Beuers U. Role of Fas and caspase 8 in glycochenodeoxycholic acid induced hepatocellular apoptosis in human liver cell lines. Falk Symposium 155. XIII FALK LIVER WEEK (Part I) XIX International Bile Acid Meeting. Bile Acids: Biological Actions and Clinical Relevance, October 6 - 7, 2006 Freiburg (Germany), p. 59 (2006). *

• Rutherford AE, Pratt DS. Cholestasis and cholestatic syndromes. Curr. Opin. Gastroenterol. 22: 209-214 (2006). *

• Saha B, Nandi D. Farnesyltransferase inhibitors reduce Ras activation and ameliorate acetaminophen-induced liver injury in mice. Hepatology 50: 1547-1557 (2009).

• Sahdev S, Saini KS, Hasnain SE. Baculovirus P35 protein: an overview of its applications across multiple therapeutic and biotechnological arenas. Biotechnol. Prog. Oct 14 [Epub ahead of print] (2009).

• Sakauchi F, Mori M, Zeniya M, Toda G. Antimitochondrial antibody negative primary biliary cirrhosis in Japan: utilization of clinical data when patients applied to receive public financial aid. J. Epidemiol. 16: 30-34 (2006). *

• Sakauchi F, Mori M, Zeniya M, Toda G. A cross-sectional study of primary biliary cirrhosis in Japan: utilization of clinical data when patients applied to receive public financial aid. J. Epidemiol. 15: 24-28 (2005). *

• Sakauchi F, Oura A, Ohnishi H, Mori M. Comparison of the clinical features of Japanese patients with primary biliary cirrhosis in 1999 and 2004: utilization of clinical data when patients applied to receive public financial aid. J. Epidemiol. 17: 210-214 (2007). *

• Sakly W, Jeddi M, Ghedira I. Anti-Saccharomyces cerevisiae antibodies in primary biliary cirrhosis. Dig. Dis. Sci. 53: 1983-1987 (2007). *

• Sakugawa H, Nakasone H, Nakayoshi T, Yamashiro T, Maeshiro T, Kobashigawa K, Kinjo F, Saito A, Zukeran H, Nakanuma Y, Ohba K. Epidemiology of primary biliary cirrhosis among women with elevated gamma-glutamyl transpeptidase levels in Okinawa, Japan. Hepatol. Res. 26: 330-336 (2003).

• Salas JT, Banales JM, Sarvide S, Recalde S, Ferrer A, Uriarte I, Oude Elferink RP, Prieto J, Medina JF. Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. Gastroenterology 134: 1482-1493 (2008). *

• Salo J, Caballeria L, Bruguera M, Pares A, Rodes J. Autoimmune cholangitis or primary biliary cirrhosis without antimitochondrial antibodies? Gastroenterol. Hepatol. 20: 5-10 (1997).

• Sanchez Hellín V, Gutierrez Rodero F. Toxicogenetics of antiretroviral treatment (II): neurotoxicity, hepatotoxicity, lactic acidosis, kidney damage, and other adverse effects of antiretroviral drugs. Enferm. Infecc. Microbiol. Clin. 26 Suppl. 6: 24-33 (2008).

• Sanchez-Munoz D, Castellano-Megias VM, Romero-Gomez M. Expression of bcl-2 in ductular proliferation is related to periportal hepatic stellate cell activation and fibrosis progression in patients with autoimmune cholestasis. Dig. Liver Dis. 39: 262-266 (2007).

• Sanchez-Pobre P, Castellano G, Colina F, Dominguez P, Rodriguez S, Canga F, Herruzo JA. Antimitochondrial antibody-negative chronic nonsuppurative destructive cholangitis. Atypical primary biliary cirrhosis or autoimmune cholangitis? J. Clin. Gastroenterol. 23: 191-198 (1996).

• Sanchez-Pobre P, Gonzalez C, Paz E, Colina F, Castellano G, Munoz-Yague T, Rodriguez S, Yela C, Alvarez V, Solis-Herruzo J. Chronic hepatitis C and autoimmune cholangitis: a case study and literature review. Dig. Dis. Sci. 47: 1224-1229 (2002). *

• Sanchez-Pobre P, Solis-Herruzo J. Autoimmune cholangitis, nosological location. Rev. Esp. Enferm. Dig. 95: 791-794 (2003).

• Sano M, Izumi Y, Helenius K, Asakura M, Rossi DJ, Xie M, Taffet G, Hu L, Pautler RG, Wilson CR, Boudina S, Abel ED, Taegtmeyer H, Scaglia F, Graham BH, Kralli A, Shimizu N, Tanaka H, Makela TP, Schneider MD. Menage-a-trois 1 is critical for the transcriptional function of PPARgamma coactivator 1. Cell. Metab. 5: 129-142 (2007). *

• Sapey T, Turlin B, Canva-Delcambre V, Mendler MH, Deugnier Y, Brissot P, Guyader D. Importance of liver puncture biopsy and endoscopic retrograde cholangiography in patients with chronic anicteric unexplained cholestasis. A retrospective study in 79 patients. Gastroenterol. Clin. Biol. 23: 178-185 (1999).

• Saraswathy S, Rao NA. Photoreceptor mitochondrial oxidative stress in experimental autoimmune uveitis. Ophthalmic Res. 40: 160-164 (2008).

• Sarin SK, Monga R, Sandhu BS, Sharma BC, Sakhuja P, Malhotra V. Primary biliary cirrhosis in India. Hepatobiliary Pancreat. Dis. Int. 5: 105-109 (2006). *

• Sasaki M, Allina J, Odin JA, Thung SN, Coppel R, Nakanuma Y, Gershwin ME. Autoimmune cholangitis in the SJL/J mouse is antigen non-specific. Dev. Immunol. 9: 103-111 (2002). *

• Sasaki M, Ansari A, Pumford N, van de Water J, Leung PS, Humphries KM, Szweda LI, Nakanuma Y, Roche TE, Coppel RL, Bach JF, Gershwin ME. Comparative immunoreactivity of anti-trifluoroacetyl (TFA) antibody and anti-lipoic acid antibody in primary biliary cirrhosis: searching for a mimic. J. Autoimmun. 15: 51-60 (2000). *

• Sasaki M, Ansari AA, Nakanuma Y, Coppel RL, Keeffe EB, Gershwin ME. The immunopathology of primary biliary cirrhosis: thoughts for the millennium. Arch. Immunol. Ther. Exp. (Warsz.) 48: 1-10 (2000). *

• Sasaki M, Van De Water J, Kenny TP, Gallo ML, Leung PS, Nakanuma Y, Ansari AA, Coppel RL, Neuberger J, Gershwin ME. Immunoglobulin gene usage and immunohistochemical characteristics of human monoclonal antibodies to the mitochondrial autoantigens of primary biliary cirrhosis induced in the XenoMouse. Hepatology 34: 631-637 (2001). *

• Sato N, Ohira H, Orikasa H, Shinzawa J, Suzuki T, Tojo J, Miyata M, Nishimaki T, Kasukawa R. Primary biliary cirrhosis associated with painless thyroiditis. Intern. Med. 38: 244-248 (1999). *

• Sato Y, Harada K, Sudo Y, Watanabe K, Nakahama T, Morimoto H, Nakanuma Y. Autoimmune hepatitis associated with bile duct injury resembling chronic non-suppurative destructive cholangitis. Pathol. Int. 52: 478-482 (2002). *

• Satoh T, Hirakata M, Yoshida T, Matsumura M, Miyachi K, Mimori T, Akizuki M. Systemic lupus erythmatosus associated with autoimmune hepatitis two cases with novel autoantibodies to transfer RNA-related antigens. Clin. Rheumatol. 16: 305-309 (1997).

• Sayers T, Leoutsakos A, Berg P, Baum H. Antimitochondrial antibodies (AMA) in primary biliary cirrhosis. I. Separation of the PBC antigen activity from mitochondrial ATPase activity. J. Bioenerg. Biomembr. 13: 255-267 (1981).

• Sayers TJ, Binder T, Berg PA. Heterogeneity of anti-mitochondrial antibodies: characterization and separation of the antigen associated with the pseudolupus erythematosus syndrome. Clin. Exp. Immunol. 37: 68-75 (1979). *

• Scealy M, Mackay IR, Rowley MJ. Amino acids critical for binding of autoantibody to an immunodominant conformational epitope of the pyruvate dehydrogenase complex subunit E2: identification by phage display and site-directed mutagenesis. Mol. Immunol. 43: 745-753 (2006). *

• Schattner A, Kaburaki Y, Kaplan MM, Miller KB. Anti-DNA antibody idiotypes in primary biliary cirrhosis. Cell. Immunol. 104: 37-46 (1987).

• Schley PD, Jijon HB, Robinson LE, Field CJ. Mechanisms of omega-3 fatty acid-induced growth inhibition in MDA-MB-231 human breast cancer cells. Breast Cancer Res. Treat. 92: 187-195 (2005).

• Schmidt G, Kienle J, Schmidt DS, Brunner G. Frequency of chronic non-suppurative destructive cholangitis and primary biliary cirrhosis in comparison with other chronic inflammatory liver diseases. Dtsch. Med. Wochenschr. 101: 693-696 (1976).

• Schmit P, Gilson G, Humbel RL. Evaluation of an automated enzyme inhibition assay for the detection of anti-mitochondrial M2 autoantibodies. Clin. Chem. 45: 2287-2289 (1999). *

• Schoemaker MH, Gommans WM, de la Rosa LC, Homan M, Klok P, Trautwein C, van Goor H, Poelstra K, Haisma HJ, Jansen PL, Moshage H. Resistance of rat hepatocytes against bile acid-induced apoptosis in cholestatic liver injury is due to nuclear factor-kappa B activation. J. Hepatol. 39: 153-161 (2003). *

• Scholz H, Lund T, Dahle MK, Collins JL, Korsgren O, Wang JE, Foss A. The synthetic liver X receptor agonist GW3965 reduces tissue factor production and inflammatory responses in human islets in vitro. Diabetologia 52: 1352-1362 (2009). *

• Schoonjans K, Staels B, Auwerx J. Role of the peroxisome proliferator-activated receptor (PPAR) in mediating the effects of fibrates and fatty acids on gene expression. J. Lipid Res. 37: 907-925 (1996). *

• Schramm C, Herkel J, Beuers U, Kanzler S, Galle PR, Lohse AW. Pregnancy in autoimmune hepatitis: outcome and risk factors. Am. J. Gastroenterol. 101: 556-560 (2006). *

• Schreibman I, Regev A. Recurrent primary biliary cirrhosis after liver transplantation - the disease and its management. MedGenMed. 8: 30 (2006). *

• Schultheiss HP, Berg P, Klingenberg M. The mitochondrial adenine nucleotide translocator is an antigen in primary biliary cirrhosis. Clin. Exp. Immunol. 54: 648-654 (1983). *

• Schultheiss HP, Berg PA, Klingenberg M. Inhibition of the adenine nucleotide translocator by organ specific autoantibodies in primary biliary cirrhosis. Clin. Exp. Immunol. 58: 596-602 (1984). *

• Schungel S, Buitrago-Molina LE, Nalapareddy PD, Lebofsky M, Manns MP, Jaeschke H, Gross A, Vogel A. The strength of the Fas ligand signal determines whether hepatocytes act as type 1 or type 2 cells in murine livers. Hepatology 50: 1558-1566 (2009).

• Schuppan D, Jia JD, Brinkhaus B, Hahn EG. Herbal products for liver diseases: a therapeutic challenge for the new millennium. Hepatology 30: 1099-1104 (1999). *

• Sebagh M, Farges O, Dubel L, Samuel D, Bismuth H, Reynes M. Histological features predictive of recurrence of primary biliary cirrhosis after liver transplantation. Transplantation 65: 1328-1333 (1998).

• Sedlack RE, Smyrk TC, Czaja AJ, Talwalkar JA. Celiac disease-associated autoimmune cholangitis. Am. J. Gastroenterol. 97: 3196-3198 (2002). *

• Seinturier C, Imbert B, Ponard D, Drouet C, Debru JL. Sneddon syndrome with anti-mitochondrial type M5 antibodies. Presse. Med. 34: 1011-1012 (2005).

• Seitz HK, Stickel F. Risk factors and mechanisms of hepatocarcinogenesis with special emphasis on alcohol and oxidative stress. Biol. Chem. 387: 349-360 (2006). *

• Seki S, Matsui T, Nakajima S, Kawai K, Monna T, Yamamoto S. Production of histological changes mimicking primary biliary cirrhosis in rabbits by immunization with bile duct antigen. Gastroenterol. Jpn. 21: 349-356 (1986).

• Sellami MK, Zitouni M, Zouiten F, Laadhar L, Ben Chaabane T, Makni S. Tuberculosis, primary biliary cirrhosis and autoimmunity (apropos of a case in Tunisia). Bull. Soc. Pathol. Exot. 94: 330-331 (2001).

• Selmi C. Environmental factors in primary biliary cirrhosis. Hepatol. Res. 37 Suppl. 3: S370-S376 (2007). *

• Selmi C, Balkwill DL, Invernizzi P, Ansari AA, Coppel RL, Podda M, Leung PS, Kenny TP, Van De Water J, Nantz MH, Kurth MJ, Gershwin ME. Patients with primary biliary cirrhosis react against a ubiquitous xenobiotic-metabolizing bacterium. Hepatology 38: 1250-1257 (2003). *

• Selmi C, Ichiki Y, Invernizzi P, Podda M, Gershwin ME. The enigma of primary biliary cirrhosis. Clin. Rev. Allergy Immunol. 28: 73-82 (2005). *

• Selmi C, Invernizzi P, Keefe EB, Coppel RL, Podda M, Rossaro L, Ansari AA, Gershwin ME. Epidemiology and pathogenesis of primary biliary cirrhosis. J. Clin. Gastroenterol. 38: 264-271 (2004).

• Selmi C, Invernizzi P, Zuin M, Podda M, Seldin MF, Gershwin ME. Genes and (auto)immunity in primary biliary cirrhosis. Genes Immun. 6: 543-556 (2005). *

• Selmi C, Lleo A, Invernizzi P, Gershwin ME. Chapter 18. Primary biliary cirrhosis and autoimmune cholangitis. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 235-248 (2007). *

• Selmi C, Lleo A, Pasini S, Zuin M, Gershwin ME. Innate immunity and primary biliary cirrhosis. Curr. Mol. Med. 9: 45-51 (2009).

• Selmi C, Ross SR, Ansari AA, Invernizzi P, Podda M, Coppel RL, Gershwin ME. Lack of immunological or molecular evidence for a role of mouse mammary tumor retrovirus in primary biliary cirrhosis. Gastroenterology 127: 493-501 (2004). *

• Selmi C, Zuin M, Bowlus CL, Gershwin ME. Anti-mitochondrial antibody-negative primary biliary cirrhosis. Clin. Liver Dis. 12: 173-185 (2008).

• Selmi C, Zuin M, Gershwin ME. The unfinished business of primary biliary cirrhosis. J. Hepatol. 49: 451-460 (2008). *

• Sema K, Takei M, Uenogawa K, Horikoshi A, Hosokawa Y, Matsuda M, Henmi A, Sawada S. Felty's syndrome with chronic hepatitis and compatible autoimmune hepatitis: a case presentation. Intern. Med. 44: 335-341 (2005). *

• Seo S, Toutounjian R, Conrad A, Blatt L, Tong MJ. Favorable outcomes of autoimmune hepatitis in a community clinic setting. J. Gastroenterol. Hepatol. 23: 1410-1414 (2008). *

• Serkova NJ, Jackman M, Brown JL, Liu T, Hirose R, Roberts JP, Maher JJ, Niemann CU. Metabolic profiling of livers and blood from obese Zucker rats. J. Hepatol. 44: 956-962 (2006). *

• Seth RB, Sun L, Chen ZJ. Antiviral innate immunity pathways. Cell Res. 16: 141-147 (2006). *

• Shan P, Yuan A, Shen L, Shen L, Chai D, Pu J, He B. RXR agonists inhibit oxidative stress-induced apoptosis in H9c2 rat ventricular cells. Biochem. Biophys. Res. Commun. 375: 628-633 (2008). *

• Shaoul R, Lerner A. Associated autoantibodies in celiac disease. Autoimmun. Rev. 6: 559-565 (2007).

• Sharma A, Provenzale D, McKusick A, Kaplan MM. Interstitial pneumonitis after low-dose methotrexate therapy in primary biliary cirrhosis. Gastroenterology 107: 266-270 (1994).

• Shen C, Assche GV, Colpaert S, Maerten P, Geboes K, Rutgeerts P, Ceuppens JL. Adalimumab induces apoptosis of human monocytes: a comparative study with infliximab and etanercept. Aliment. Pharmacol. Ther. 21: 251-258 (2005). *

• Shen M, Zhang F, Zhang X. Primary biliary cirrhosis complicated with interstitial lung disease: a prospective study in 178 patients. J. Clin. Gastroenterol. 43: 676-679 (2009).

• Sherlock S. Primary biliary cirrhosis: critical evaluation and treatment policies. Scand. J. Gastroenterol. Suppl. 77: 63-74 (1982).

• Sherlock S. Primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune cholangitis. Clin. Liver Dis. 4: 97-113 (2000).

• Sherlock S. Ludwig Symposium on biliary disorders. Autoimmune cholangitis: a unique entity? Mayo Clin. Proc. 73: 184-190 (1998). *

• Sherman EA, Strauss KA, Tortorelli S, Bennett MJ, Knerr I, Morton DH, Puffenberger EG. Genetic mapping of glutaric aciduria, type 3, to chromosome 7 and identification of mutations in C7orf10. Am. J. Hum. Genet. 83: 604-609 (2008). *

• Sheu KR, Blass JP. The alpha-ketoglutarate dehydrogenase complex. Ann. N. Y. Acad. Sci. 893: 61-78 (1999).

• Shi X, Zhang C, Jia J, Zhang F, Qian L, Wang B. Histopathological features of 27 cases of primary biliary cirrhosis. Zhonghua Gan Zang Bing Za Zhi 10: 338-340 (2002).

• Shibasaki T, Shimada T, Morita T, Sakai O. Immunogenetical penetrance in patients with primary biliary cirrhosis. Jpn. J. Med. 29: 533-536 (1990).

• Shibata J, Fujiyama S, Honda Y, Sato T. Combination therapy with ursodeoxycholic acid and colchicine for primary biliary cirrhosis. J. Gastroenterol. Hepatol. 7: 277-282 (1992).

• Shibata M, Morizane T, Tanaka A, Onozuka Y, Uchida T, Gershwin ME, Mitamura K. A case of autoimmune hepatitis with a high titer of antimitochondrial antibody and normal gamma-globulinemia. J. Gastroenterol. Hepatol. 16: 830-835 (2001). *

• Shibata M, Onozuka Y, Morizane T, Koizumi H, Kawaguchi N, Miyakawa H, Kako M, Mitamura K. Prevalence of antimitochondrial antibody in Japanese corporate workers in Kanagawa prefecture. J. Gastroenterol. 39: 255-259 (2004). *

• Shigematsu H, Shimoda S, Nakamura M, Matsushita S, Nishimura Y, Sakamoto N, Ichiki Y, Niho Y, Gershwin ME, Ishibashi H. Fine specificity of T cells reactive to human PDC-E2 163-176 peptide, the immunodominant autoantigen in primary biliary cirrhosis: implications for molecular mimicry and cross-recognition among mitochondrial autoantigens. Hepatology 32: 901-909 (2000). *

• Shikuwa S, Omagari K, Mizuta Y, Murase K, Osabe M, Yoshimura M, Ohfukuji M, Takehara Y, Isomoto H, Ito M, Shimokawa I, Kohno S. Primary biliary cirrhosis associated with idiopathic retroperitoneal fibrosis. J. Gastroenterol. 35: 646-648 (2000). *

• Shimoda S, Miyakawa H, Nakamura M, Ishibashi H, Kikuchi K, Kita H, Niiro H, Arinobu Y, Ono N, Mackay IR, Gershwin ME, Akashi K. CD4 T-cell autoreactivity to the mitochondrial autoantigen PDC-E2 in AMA-negative primary biliary cirrhosis. J. Autoimmun. 31: 110-115 (2008).

• Shimoda S, Nakamura M, Ishibashi H, Kawano A, Kamihira T, Sakamoto N, Matsushita S, Tanaka A, Worman HJ, Gershwin ME, Harada M. Molecular mimicry of mitochondrial and nuclear autoantigens in primary biliary cirrhosis. Gastroenterology 124: 1915-1925 (2003). *

• Shimoda S, Van de Water J, Ansari A, Nakamura M, Ishibashi H, Coppel RL, Lake J, Keeffe EB, Roche TE, Gershwin ME. Identification and precursor frequency analysis of a common T cell epitope motif in mitochondrial autoantigens in primary biliary cirrhosis. J. Clin. Invest. 102: 1831-1840 (1998). *

• Shingai R, Maeda T, Onishi S, Yamamoto Y. Autoantibody against 70 kD heat shock protein in patients with autoimmune liver diseases. J. Hepatol. 23: 382-390 (1995). *

• Shiraoku H, Hokamura M, Sagara K, Fujiyama S. A case of primary sclerosing cholangitis. Gastroenterol. Jpn. 20: 368-373 (1985).

• Shoenfeld Y, Twig G, Katz U, Sherer Y. Autoantibody explosion in antiphospholipid syndrome. J. Autoimmun. 30: 74-83 (2008).

• Siegel JL, Luthra H, Donlinger J, Angulo P, Lindor K. Association of primary biliary cirrhosis and rheumatoid arthritis. J. Clin. Rheumatol. 9: 340-343 (2003).

• Siegel SM, Selmi C, Dottorini L, Antelmi M, Zuin M, Lanfredini M, Gershwin ME. Late-onset autoimmunity: the paradigm of primary biliary cirrhosis - a mini-review. Gerontology 54: 193-201 (2008).

• Siegert W, Stemerowicz R, Hopf U. Antimitochondrial antibodies in patients with chronic graft-versus-host disease. Bone Marrow Transplant. 10: 221-227 (1992).

• Siems W, Salerno C, Crifo C, Sommerburg O, Wiswedel I. beta-Carotene degradation products - formation, toxicity and prevention of toxicity. Forum Nutr. 61: 75-86 (2009).

• Silvagno F, De Vivo E, Attanasio A, Gallo V, Mazzucco G, Pescarmona G. Mitochondrial localization of vitamin D receptor in human platelets and differentiated megakaryocytes. PLoS One 5: e8670 (2010). *

• Silver PB, Agarwal RK, Su SB, Suffia I, Grajewski RS, Luger D, Chan CC, Mahdi RM, Nickerson JM, Caspi RR. Hydrodynamic vaccination with DNA encoding an immunologically privileged retinal antigen protects from autoimmunity through induction of regulatory T cells. J. Immunol. 179: 5146-5158 (2007). *

• Sinclair D, Spedding A, Young R. Can the laboratory affect the investigation and diagnosis of primary biliary cirrhosis? J. Clin. Pathol. 59: 360-362 (2006). *

• Singh G, Palaniappan S, Rotimi O, Hamlin PJ. Autoimmune hepatitis triggered by hepatitis A. Gut 56: 304 (2007). *

• Singh R, Wang Y, Xiang Y, Tanaka KE, Gaarde WA, Czaja MJ. Differential effects of JNK1 and JNK2 inhibition on murine steatohepatitis and insulin resistance. Hepatology 49: 87-96 (2009). *

• Sinitsyna O, Krysanova Z, Ishchenko A, Dikalova AE, Stolyarov S, Kolosova N, Vasunina E, Nevinsky G. Age-associated changes in oxidative damage and the activity of antioxidant enzymes in rats with inherited overgeneration of free radicals. J. Cell. Mol. Med. 10: 206-215 (2006). *

• Siordia-Reyes AG, Ferman-Cano F, Garcia GR, Rodriguez-Velasco A. Wilson disease. Report of a case of autopsy with copper tissue quantification and electronic microscopy. Rev. Gastroenterol. Mex. 66: 38-41 (2001).

• Siqueira MF, Li J, Chehab L, Desta T, Chino T, Krothpali N, Behl Y, Alikhani M, Yang J, Braasch C, Graves DT. Impaired wound healing in mouse models of diabetes is mediated by TNF-alpha dysregulation and associated with enhanced activation of forkhead box O1 (FOXO1). Diabetologia Nov 10 [Epub ahead of print] (2009). *

• Skopouli FN, Barbatis C, Moutsopoulos HM. Liver involvement in primary Sjogren's syndrome. Br. J. Rheumatol. 33: 745-748 (1994).

• Smith ND, Boyer JL. Are antimitochondrial antibodies of prognostic value in primary biliary cirrhosis? Hepatology 6: 739-741 (1986).

• Soden JS, Devereaux MW, Haas JE, Gumpricht E, Dahl R, Gralla J, Traber MG, Sokol RJ. Subcutaneous vitamin E ameliorates liver injury in an in vivo model of steatocholestasis. Hepatology 46: 485-495 (2007). *

• Sohda T, Shiga H, Nakane H, Nishizawa S, Yoshikane M, Anan A, Suzuki N, Irie M, Iwata K, Watanabe H, Sakisaka S. Rapid-onset primary biliary cirrhosis resembling drug-induced liver injury. Intern. Med. 44: 1051-1054 (2005). *

• Sokol RJ, Dahl R, Devereaux MW, Yerushalmi B, Kobak GE, Gumpricht E. Human hepatic mitochondria generate reactive oxygen species and undergo the permeability transition in response to hydrophobic bile acids. J. Pediatr. Gastroenterol. Nutr. 41: 235-243 (2005). *

• Sokol RJ, Straka MS, Dahl R, Devereaux MW, Yerushalmi B, Gumpricht E, Elkins N, Everson G. Role of oxidant stress in the permeability transition induced in rat hepatic mitochondria by hydrophobic bile acids. Pediatr. Res. 49: 519-531 (2001). *

• Sokol RJ, Winklhofer-Roob BM, Devereaux MW, McKim JM Jr. Generation of hydroperoxides in isolated rat hepatocytes and hepatic mitochondria exposed to hydrophobic bile acids. Gastroenterology 109: 1249-1256 (1995). *

• Solazzo M, Fantappie O, Lasagna N, Sassoli C, Nosi D, Mazzanti R. P-gp localization in mitochondria and its functional characterization in multiple drug-resistant cell lines. Exp. Cell Res. 312: 4070-4078 (2006). *

• Solis Herruzo JA, Solis Munoz P, Munoz Yague T. The pathogenesis of primary biliary cirrhosis. Rev. Esp. Enferm. Dig. 101: 413-423 (2009). *

• Song KH, Lee WJ, Koh JM, Kim HS, Youn JY, Park HS, Koh EH, Kim MS, Youn JH, Lee KU, Park JY. alpha-Lipoic acid prevents diabetes mellitus in diabetes-prone obese rats. Biochem. Biophys. Res. Commun. 326: 197-202 (2005). *

• Soucek K, Pachernik J, Kubala L, Vondracek J, Hofmanova J, Kozubik A. Transforming growth factor-beta1 inhibits all-trans retinoic acid-induced apoptosis. Leuk. Res. 30: 607-623 (2006).

• Souid AK, Penefsky HS, Sadowitz PD, Toms B. Enhanced cellular respiration in cells exposed to doxorubicin. Mol. Pharm. 3: 307-321 (2006). *

• Soulie E, Moulin V, Fourel V, Dubel L, Kantara-Doukoure M, Johanet C. Characterization of anti-mitochondrial antibodies type 2 (anti-M2): comparison of two techniques, western blotting and indirect immunofluorescence. Ann. Biol. Clin. (Paris) 51: 707-711 (1993).

• Sparso T, Hussain MS, Andersen G, Hainerova I, Borch-Johnsen K, Jorgensen T, Hansen T, Pedersen O. Relationships between the functional PPARalpha Leu162Val polymorphism and obesity, type 2 diabetes, dyslipidaemia, and related quantitative traits in studies of 5799 middle-aged white people. Mol. Genet. Metab. 90: 205-209 (2007). *

• Speiciene D, Irnius A, Leuschner U, Liakina V, Semuchiniene T, Barakauskiene A. Primary biliary cirrhosis in Lithuania: diagnosis and clinical picture. Med. Sci. Monit. 13: CR299-CR306 (2007). *

• Springer J, Cauch-Dudek K, O'Rourke K, Wanless IR, Heathcote EJ. Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis. Am. J. Gastroenterol. 94: 47-53 (1999). *

• Stachowska E, Baskiewicz-Masiuk M, Dziedziejko V, Gutowska I, Baranowska-Bosiacka I, Marchlewicz M, Dolegowska B, Wiszniewska B, Machalinski B, Chlubek D. Conjugated linoleic acid increases intracellular ROS synthesis and oxygenation of arachidonic acid in macrophages. Nutrition 24: 187-199 (2008). *

• Stanca CM, Fiel MI, Allina J, Caracta CF, Odin JA. Liver failure in an antimitochondrial antibody-positive patient with sarcoidosis: primary biliary cirrhosis or hepatic sarcoidosis? Semin. Liver Dis. 25: 364-370 (2005).

• Stankov MV, Lucke T, Das AM, Schmidt RE, Behrens GM. Mitochondrial DNA depletion and respiratory chain activity in primary human subcutaneous adipocytes treated with nucleoside-analogue reverse transcriptase inhibitors. Antimicrob. Agents Chemother. 54: 280-287 (2010). *

• Stechemesser E, Klein R, Berg PA. Characterization and clinical relevance of liver-pancreas antibodies in autoimmune hepatitis. Hepatology 18: 1-9 (1993).

• Steele IL, Levy C, Lindor KD. Primary sclerosing cholangitis - approach to diagnosis. MedGenMed. 9: 20 (2007). *

• Stemerowicz R, Hopf U, Moller B, Wittenbrink C, Rodloff A, Reinhardt R, Freudenberg M, Galanos C. Are antimitochondrial antibodies in primary biliary cirrhosis induced by R(rough)-mutants of Enterobacteriaceae? Lancet 2: 1166-1170 (1988).

• Stojanovic J, Srefanovic D, Vulovic D, Puskas L, Markovic L. Role of apoptosis in pathogenesis of thyroiditis. Med. Pregl. 62: 49-52 (2009).

• Stone J, Wade JA, Cauch-Dudek K, Ng C, Lindor KD, Heathcote EJ. Human leukocyte antigen class II associations in serum antimitochondrial antibodies (AMA)-positive and AMA-negative primary biliary cirrhosis. J. Hepatol. 36: 8-13 (2002). *

• Storch W. Diagnostic significance of antibodies against cell organelles (mitochondria, endoplasmic reticulum and ribosomes). Schweiz. Med. Wochenschr. 113: 47-59 (1983).

• Strassburg CP. Autoimmune liver diseases. Falk Symposium 146: GUT-LIVER INTERACTIONS: BASIC AND CLINICAL CONCEPTS, Innsbruck (Austria), March 11-12, 2005, pp. 66-70 (2005). *

• Strassburg CP. Autoimmune liver diseases and their overlap syndromes. Schweiz. Rundsch. Med. Prax. 95: 1363-1381 (2006).

• Strassburg CP. Molecular characterization of autoantigens in liver diseases. Falk Symposium 135. Immmunological diseases of liver and gut. Prague (Czech Republic). p. 30. September 12 - 13 (2003). *

• Strassburg CP, Jaeckel E, Manns MP. Anti-mitochondrial antibodies and other immunological tests in primary biliary cirrhosis. Eur. J. Gastroenterol. Hepatol. 11: 595-601 (1999).

• Strassburg CP, Manns MP. Chapter 8. Prevalence and significance of autoantibodies in acute and chronic liver diseases and hepatocellular carcinoma. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 95-110 (2007). *

• Strassburg CP, Manns MP. Autoimmune tests in primary biliary cirrhosis. Best Pract. Res. Clin. Gastroenterol. 14: 585-599 (2000). *

• Strassburg CP, Manns MP. Primary biliary cirrhosis and overlap syndromes. Diagnosis and therapy. Internist (Berl.) 45: 16-26 (2004).

• Strauss G, Westhoff MA, Fischer-Posovszky P, Fulda S, Schanbacher M, Eckhoff SM, Stahnke K, Vahsen N, Kroemer G, Debatin KM. 4-Hydroperoxy-cyclophosphamide mediates caspase-independent T-cell apoptosis involving oxidative stress-induced nuclear relocation of mitochondrial apoptogenic factors AIF and EndoG. Cell Death Differ. 15: 332-343 (2008). *

• Stravitz RT, Vlahcevic ZR, Russell TL, Heizer ML, Avadhani NG, Hylemon PB. Regulation of sterol 27-hydroxylase and an alternative pathway of bile acid biosynthesis in primary cultures of rat hepatocytes. J. Steroid Biochem. Mol. Biol. 57: 337-347 (1996).

• Styles NA, Falany JL, Barnes S, Falany CN. Quantification and regulation of the subcellular distribution of bile acid CoA:amino acid N-acyltransferase activity in rat liver. J. Lipid Res. 48: 1305-1315 (2007). *

• Su CW, Hung HH, Huo TI, Huang YH, Li CP, Lin HC, Lee PC, Lee SD, Wu JC. Natural history and prognostic factors of primary biliary cirrhosis in Taiwan: a follow-up study up to 18 years. Liver Int. 28: 1305-1313 (2008). *

• Su CW, Wu JC, Chiou YY, Tsay SH, Huo TI, Huang YH, Hung HH, Lin HC, Lee SD. Successful radiofrequency ablation therapy for hepatocellular carcinoma in a male patient with early stage primary biliary cirrhosis and positive serum hepatitis B core antibody. J. Chin. Med. Assoc. 71: 40-44 (2008).

• Subasic D, Karamehic J, Ljuca F, Gavrankapetanovic F, Delic-Sarac M, Eminovic I, Kovacevic D. Correlation of autoantibodies presence detected by IFA-anti-dsDNA, IFA-AMA and immunoblotting with corresponding data in clinical management of autoimmune diseases. Bosn. J. Basic Med. Sci. 8: 86-92 (2008).

• Sundberg TB, Swensen L, Wahl D, Opipari AW, Glick GD. Apoptotic signaling activated by modulation of the FoF1-ATPase: implications for selective killing of autoimmune lymphocytes. J. Pharmacol. Exp Ther. 331: 437-444 (2009). *

• Sundewall AC, Lefvert AK, Norberg R. Characterization of anti-acetylcholine receptor antibody activity in patients with anti-mitochondrial antibodies. Clin. Immunol. Immunopathol. 45: 184-195 (1987).

• Sundin U. Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis. Clin. Exp. Immunol. 81: 238-243 (1990). *

• Surh CD, Ahmed-Ansari A, Gershwin ME. Comparative epitope mapping of murine monoclonal and human autoantibodies to human PDH-E2, the major mitochondrial autoantigen of primary biliary cirrhosis. J. Immunol. 144: 2647-2652 (1990).

• Surh CD, Cooper AE, Coppel RL, Leung P, Ahmed A, Dickson R, Gershwin ME. The predominance of IgG3 and IgM isotype antimitochondrial autoantibodies against recombinant fused mitochondrial polypeptide in patients with primary biliary cirrhosis. Hepatology 8: 290-295 (1988).

• Surh CD, Coppel R, Gershwin ME. Structural requirement for autoreactivity on human pyruvate dehydrogenase-E2, the major autoantigen of primary biliary cirrhosis. Implication for a conformational autoepitope. J. Immunol. 144: 3367-3374 (1990).

• Surh CD, Danner DJ, Ahmed A, Coppel RL, Mackay IR, Dickson ER, Gershwin ME. Reactivity of primary biliary cirrhosis sera with a human fetal liver cDNA clone of branched-chain alpha-keto acid dehydrogenase dihydrolipoamide acyltransferase, the 52 kD mitochondrial autoantigen. Hepatology 9: 63-68 (1989).

• Surh CD, Roche TE, Danner DJ, Ansari A, Coppel RL, Prindiville T, Dickson ER, Gershwin ME. Antimitochondrial autoantibodies in primary biliary cirrhosis recognize cross-reactive epitope(s) on protein X and dihydrolipoamide acetyltransferase of pyruvate dehydrogenase complex. Hepatology 10: 127-133 (1989).

• Sutter AP, Maaser K, Hopfner M, Huether A, Schuppan D, Scherubl H. Cell cycle arrest and apoptosis induction in hepatocellular carcinoma cells by HMG-CoA reductase inhibitors. Synergistic antiproliferative action with ligands of the peripheral benzodiazepine receptor J. Hepatol. 43: 808-816 (2005). *

• Suwa M, Egashira T, Nakano H, Sasaki H, Kumagai S. Metformin increases the PGC-1alpha protein and oxidative enzyme activities possibly via AMPK phosphorylation in skeletal muscle in vivo. J. Appl. Physiol. 101: 1685-1692 (2006). *

• Suzuki A, Van de Water J, Gershwin ME, Jorgensen R, Angulo P, Lindor K. Oral tolerance and pyruvate dehydrogenase in patients with primary biliary cirrhosis. Dev. Immunol. 9: 55-61 (2002). *

• Suzuki Y, Arase Y, Ikeda K, Saitoh S, Tsubota A, Suzuki F, Kobayashi M, Akuta N, Someya T, Miyakawa Y, Kumada H. Clinical and pathological characteristics of the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. J. Gastroenterol. Hepatol. 19: 699-706 (2004). *

• Swana GT, Swana MR, Bottazzo GF, Doniach D. A human-specific mitochondrial antibody its importance in the identification of organ-specific reactions. Clin. Exp. Immunol. 28: 517-525 (1977). *

• Taal BG, Schalm SW, ten Kate FW, Hermans J, Geertzen RG, Feltkamp BE. Clinical diagnosis of primary biliary cirrhosis: a classification based on major and minor criteria. Hepatogastroenterology 30: 178-182 (1983).

• Tahara K, Yukawa S, Shoji A, Hayashi H, Tsuboi N. Long-term remission by cyclosporine in a patient with eosinophilic fasciitis associated with primary biliary cirrhosis. Clin. Rheumatol. 27: 1199-1201 (2008). *

• Tak WY, Park SY, Jung MK, Jeon SW, Cho CM, Kweon YO, Kim SK, Choi YH. Mitochondrial myopathy caused by clevudine therapy in chronic hepatitis B patients. Hepatol. Res. 39: 944-947 (2009). *

• Takahashi T, Honma T, Ishizuka K, Fuse I, Asakura H. A female with asymptomatic primary biliary cirrhosis associated with pernicious anemia. J. Gastroenterol. Hepatol. 16: 1420-1424 (2001). *

• Take H, Kubota K, Tamura K, Kurabayashi H, Tamura J, Shirakura T. Acquired total immunoglobulin G deficiency in a patient with primary biliary cirrhosis. J. Intern. Med. 238: 289-292 (1995).

• Takeda I, Igarashi S, Oyanagi H, Irisawa A, Mukai S, Shoji I, Sakuma H, Satho H, Sekine K, Kuroda M, et al. Two cases of asymptomatic primary biliary cirrhosis (PBC) accompanied with CREST syndrome. Ryumachi 34: 767-772 (1994).

• Takehara T, Tatsumi T, Suzuki T, Rucker EB, Hennighausen L, Jinushi M, Miyagi T, Kanazawa Y, Hayashi N. Hepatocyte-specific disruption of Bcl-xL leads to continuous hepatocyte apoptosis and liver fibrotic responses. Gastroenterology 127: 1189-1197 (2004). *

• Tal MC, Iwasaki A. Autophagic control of RLR signaling. Autophagy 5: 749-750 (2009).

• Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet 362: 53-61 (2003). *

• Tamura H, Okamoto M, Yamashita T, Sato C, Watanabe A, Kondo A, Tatsuguchi A, Tsuji T, Ogata K, Dan K. Pure white cell aplasia: report of the first case associated with primary biliary cirrhosis. Int. J. Hematol. 85: 97-100 (2007).

• Tan KP, Kosuge K, Yang M, Ito S. NRF2 as a determinant of cellular resistance in retinoic acid cytotoxicity. Free Radic. Biol. Med. 45: 1663-1673 (2008).

• Tanaka A, Borchers AT, Ishibashi H, Ansari AA, Keen CL, Gershwin ME. Genetic and familial considerations of primary biliary cirrhosis. Am. J. Gastroenterol. 96: 8-15 (2001). *

• Tanaka A, Miyakawa H, Luketic VA, Kaplan M, Storch WB, Gershwin ME. The diagnostic value of anti-mitochondrial antibodies, especially in primary biliary cirrhosis. Cell Mol. Biol. (Noisy-le-grand) 48: 295-299 (2002).

• Tanaka A, Nalbandian G, Leung PS, Benson GD, Munoz S, Findor JA, Branch AD, Coppel RL, Ansari AA, Gershwin ME. Mucosal immunity and primary biliary cirrhosis: presence of antimitochondrial antibodies in urine. Hepatology 32: 910-915 (2000). *

• Tanaka A, Nezu S, Uegaki S, Kikuchi K, Shibuya A, Miyakawa H, Takahashi S, Bianchi I, Zermiani P, Podda M, Ohira H, Invernizzi P, Takikawa H. Vitamin D receptor polymorphisms are associated with increased susceptibility to primary biliary cirrhosis in Japanese and Italian populations. J. Hepatol. 50: 1202-1209 (2009). *

• Tanaka A, Nezu S, Uegaki S, Mikami M, Okuyama S, Kawamura N, Aiso M, Gershwin ME, Takahashi S, Selmi C, Takikawa H. The clinical significance of IgA antimitochondrial antibodies in sera and saliva in primary biliary cirrhosis. Ann. N. Y. Acad. Sci. 1107: 259-270 (2007). *

• Tanaka M, Nagai T, Tsuneyoshi Y, Sunahara N, Matsuda T, Nakamura T, Tsuyama S, Hasui K, Fitzgerald O, Matsuyama T. Expansion of a unique macrophage subset in rheumatoid arthritis synovial lining layer. Clin. Exp. Immunol. 154: 38-47 (2008). *

• Tanaka Y, Naitoh M, Yoshiura K, Ookubo K, Uegaki S, Matsumoto K, Sawabe M, Esaki Y, Kino K, Tsuru M, Fukazawa T, Takubo K. Hepatocellular carcinoma arising in an elderly male with primary biliary cirrhosis. Eur. J. Gastroenterol. Hepatol. 12: 239-241 (2000).

• Tanimoto H, Shimoda S, Nakamura M, Ishibashi H, Kawano A, Kamihira T, Matsushita. Promiscuous T cells selected by Escherichia coli: OGDC-E2 in primary biliary cirrhosis. J. Autoimmun. 20: 255-263 (2003). *

• Tanus T, Levinson AI, Atkins PC, Zweiman B. Polyautoimmune syndrome in common variable immunodeficiency. J. Intern. Med. 234: 525-527 (1993).

• Tarantino G, Di Minno MN, Capone D. Drug-induced liver injury: is it somehow foreseeable? World J. Gastroenterol. 15: 2817-2833 (2009). *

• Taylor JM, Borthwick F, Bartholomew C, Graham A. Over-expression of steroidogenic acute regulatory protein (StAR) increases macrophage cholesterol efflux to apolipoprotein AI. Cardiovasc. Res. Jan 18 [Epub ahead of print] (2010). *

• Taylor SL, Dean PJ, Riely CA. Primary autoimmune cholangitis. An alternative to antimitochondrial antibody-negative primary biliary cirrhosis. Am. J. Surg. Pathol. 18: 91-99 (1994).

• te Sligte K, Bourass I, Sels JP, Driessen A, Stockbrugger RW, Koek GH. Non-alcoholic steatohepatitis: review of a growing medical problem. Eur. J. Intern. Med. 15: 10-21 (2004). *

• Teoh KL, Mackay IR, Rowley MJ, Fussey SP. Enzyme inhibitory autoantibodies to pyruvate dehydrogenase complex in primary biliary cirrhosis differ for mammalian, yeast and bacterial enzymes: implications for molecular mimicry. Hepatology 19: 1029-1033 (1994).

• Teoh KL, Rowley MJ, Zafirakis H, Dickson ER, Wiesner RH, Gershwin ME, MacKay IR. Enzyme inhibitory autoantibodies to pyruvate dehydrogenase complex in primary biliary cirrhosis: applications of a semiautomated assay. Hepatology 20: 1220-1224 (1994).

• Terasaki S, Nakanuma Y, Hoso M, Ogino H, Unoura M, Kobayashi K, Mizuno Y, Nakagawa H, Shimizu M, Kanai M, et al. Three cases of primary biliary cirrhosis associated with bronchial asthma. J. Gastroenterol. 30: 667-671 (1995).

• Terjung B, Spengler U. Role of auto-antibodies for the diagnosis of chronic cholestatic liver diseases. Clin. Rev. Allergy Immunol. 28: 115-133 (2005). *

• Terracciano LM, Patzina RA, Lehmann FS, Tornillo L, Cathomas G, Mhawech P, Vecchione R, Bianchi L. A spectrum of histopathologic findings in autoimmune liver disease. Am. J. Clin. Pathol. 114: 705-711 (2000). *

• Tervaert JW, Van Hoek B, Koek G. Antineutrophil cytoplasmic antibodies in small-duct primary sclerosing cholangitis. Gastroenterology 136: 364 (2009). *

• Teubner A, Tillmann HL, Schuppan D, Gericke G, Manns MP, Stolzel U. Prevalence of circulating autoantibodies in healthy individuals. Med. Klin. (Munich) 97: 645-649 (2002).

• Thao TD, Ryu HC, Yoo SH, Rhee DK. Antibacterial and anti-atrophic effects of a highly soluble, acid stable UDCA formula in Helicobacter pylori-induced gastritis. Biochem. Pharmacol. 75: 2135-2146 (2008). *

• Thimme R, Opitz OG, Blum HE, Kreisel W. Cholestatic liver diseases. Ther. Umsch. 61: 521-527 (2004).

• Thirunavukkarasu C, Wang LF, Harvey SA, Watkins SC, Chaillet JR, Prelich J, Starzl TE, Gandhi CR. Augmenter of liver regeneration: an important intracellular survival factor for hepatocytes. J. Hepatol. 48: 578-588 (2008). *

• Tiao MM, Lin TK, Wang PW, Chen JB, Liou CW. The role of mitochondria in cholestatic liver injury. Chang Gung Med. J. 32: 346-353 (2009).

• Tison V, Callea F, Morisi C, Mancini AM, Desmet VJ. Spontaneous "primary biliary cirrhosis" in rabbits. Liver 2: 152-161 (1982).

• Toblli JE, Findor J, Sorda J, Bruch Igartua E, Hasenclever K, Collado HD. Latent distal renal tubular acidosis (dRTA) in primary biliary cirrhosis (PBC) and chronic autoimmune hepatitis (CAH). Acta Gastroenterol. Latinoam. 23: 235-238 (1993).

• Toda G, Ikeda Y, Hashimoto N, Yamazaki M, Torii M, Oka H. Liver cell membrane antibody detected by protein A and isolated rabbit liver plasma membrane in sera of patients with chronic liver diseases. Clin. Exp. Immunol. 54: 661-670 (1983). *

• Tojo J, Ohira H, Suzuki T, Takeda I, Shoji I, Kojima T, Takagi T, Kuroda M, Miyata M, Nishimaki T, Ohnami K, Kanno T, Mukai S, Kasukawa R. Clinicolaboratory characteristics of patients with primary biliary cirrhosis associated with CREST symptoms. Hepatol. Res. 22: 187-195 (2002).

• tom Dieck H, Doring F, Fuchs D, Roth HP, Daniel H. Transcriptome and proteome analysis identifies the pathways that increase hepatic lipid accumulation in zinc-deficient rats. J. Nutr. 135: 199-205 (2005). *

• Tomiyama N, Matzno S, Kitada C, Nishiguchi E, Okamura N, Matsuyama K. The possibility of simvastatin as a chemotherapeutic agent for all-trans retinoic acid-resistant promyelocytic leukemia. Biol. Pharm. Bull. 31: 369-374 (2008). *

• Tong MJ, Nies KM, Reynolds TB, Quismorio FP. Immunological studies in familial primary biliary cirrhosis. Gastroenterology 71: 305-307 (1976).

• Toyoda M, Yokomori H, Kaneko F, Yoshida H, Hoshi K, Takeuchi H, Tahara K, Takahashi A, Kudo T, Motoori T, Ohbu M, Kondo H, Hibi T. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome concomitant with systemic sclerosis, immune thrombocytopenic purpura. Intern. Med. 48: 2019-2023 (2009). *

• Trauner M, Boyer JL. Cholestatic syndromes. Curr. Opin. Gastroenterol. 20: 220-230 (2004). *

• Trautmann F, Arnold W, Manns M, Stoltenburg-Didinger G, Meyer zum Buschenfelde KH. Antimitochondrial antibodies against the M2-antigen with immune vasculitis and discoid lupus erythematosus without proof of liver disease. Z. Gastroenterol. 20: 659-666 (1982).

• Triger DR, Charlton CA, Ward AM. What does the antimitochondrial antibody mean? Gut 23: 814-818 (1982). *

• Tromm A, May B, Klein CG, Klein R, Fisseler-Eckhoff A, Griga T. Long-term response of primary biliary cirrhosis (stage I) to therapy with ursodeoxycholic acid. Hepatogastroenterology 52: 753-756 (2005).

• Tsai SL, Lai MY, Chen DS. Analysis of rearranged T cell receptor (TCR) V beta transcripts in livers of primary biliary cirrhosis: preferential V beta usage suggests antigen-driven selection. Clin. Exp. Immunol. 103: 99-104 (1996). *

• Tsaregorodtseva TM, Serova TI. Diagnostic and prognostic value of immune status indices for alimentary system diseases. Eksp. Klin. Gastroenterol. 2: 93-99, 144-145 (2007).

• Tsaregorodtseva TM, Serova TI, Vinnitskaia EV, Sabel'nikova EA, Berezina OV. Autoimmune reactions in gastrointestinal diseases. Ter. Arkh. 81: 68-71 (2009).

• Tsay GJ, Zouali M. Unscrambling the role of human parvovirus B19 signaling in systemic autoimmunity. Biochem. Pharmacol. 72: 1453-1459 (2006). *

• Tsiakalou V, Tsangaridou E, Polioudaki H, Nifli AP, Koulentaki M, Akoumianaki T, Kouroumalis E, Castanas E, Theodoropoulos PA. Optimized detection of circulating anti-nuclear envelope autoantibodies by immunofluorescence. BMC Immunol. 7: 20 (2006). *

• Tsou YK, Yeh CT. Primary biliary cirrhosis in antimitochondrial antibody-negative patients: Chang Gung Memorial Hospital experience. Chang Gung Med. J. 26: 323-329 (2003).

• Tsuboyama-Kasaoka N, Sano K, Shozawa C, Osaka T, Ezaki O. Studies of UCP2-transgenic and -knockout mice reveal that liver UCP2 is not essential for the anti-obesity effects of fish oil. Am. J. Physiol. Endocrinol. Metab. 294: E600-E606 (2008). *

• Tsuji H, Murai K, Akagi K, Fujishima M. Familial primary biliary cirrhosis associated with impaired concanavalin A-induced lymphocyte transformation in relatives. Two family studies. Dig. Dis. Sci. 37: 353-360 (1992).

• Tsuji K, Watanabe Y, Kouno H, Aisaka Y, Masanaga T, Ishihara H, Kamiyasu M, Nakanishi T, Chayama K, Asahara T, Coppel R, Gershwin ME. A case of latent primary biliary cirrhosis. Hepatol. Res. 28: 166-169 (2004).

• Tsuji K, Watanabe Y, Van De Water J, Nakanishi T, Kajiyama G, Parikh-Patel A, Coppel R, Gershwin ME. Familial primary biliary cirrhosis in Hiroshima. J. Autoimmun. 13: 171-178 (1999). *

• Tsuneyama K, Nose M, Nisihara M, Katayanagi K, Harada K, Nakanuma Y. Spontaneous occurrence of chronic non-suppurative destructive cholangitis and antimitochondrial autoantibodies in MRL/lpr mice: possible animal model for primary biliary cirrhosis. Pathol. Int. 51: 418-424 (2001). *

• Tsuneyama K, Van de Water J, Nakanuma Y, Cha S, Ansari A, Coppel R, Gershwin ME. Human combinatorial autoantibodies and mouse monoclonal antibodies to PDC-E2 produce abnormal apical staining of salivary glands in patients with coexistent primary biliary cirrhosis and Sjogren's syndrome. Hepatology 20: 893-898 (1994).

• Tsuneyama K, Van De Water J, Van Thiel D, Coppel R, Ruebner B, Nakanuma Y, Dickson ER, Gershwin ME. Abnormal expression of PDC-E2 on the apical surface of biliary epithelial cells in patients with antimitochondrial antibody-negative primary biliary cirrhosis. Hepatology 22: 1440-1446 (1995).

• Tsuzuki T, Kambe T, Shibata A, Kawakami Y, Nakagawa K, Miyazawa T. Conjugated EPA activates mutant p53 via lipid peroxidation and induces p53-dependent apoptosis in DLD-1 colorectal adenocarcinoma human cells. Biochim. Biophys. Acta 1771: 20-30 (2007). *

• Tuaillon N, Andre C, Briand JP, Penner E, Muller S. A lipoyl synthetic octadecapeptide of dihydrolipoamide acetyltransferase specifically recognized by anti-M2 autoantibodies in primary biliary cirrhosis. J. Immunol. 148: 445-450 (1992).

• Tucci P, Cione E, Perri M, Genchi G. All-trans-retinoic acid induces apoptosis in Leydig cells via activation of the mitochondrial death pathway and antioxidant enzyme regulation. J. Bioenerg. Biomembr. 40: 315-323 (2008). *

• Tucker LE. Primary biliary cirrhosis: current diagnosis and treatment. Gastroenterol. Nurs. 15: 70-76 (1992).

• Turchany JM, Leung PS, Iwayama T, Jefferson DM, Ishida J, Yamaguchi M, Munoz S, Danner DJ, Dickson ER, Gershwin ME. Comparative metabolism and structure of BCKD-E2 in primary biliary cirrhosis. J. Autoimmun. 6: 459-466 (1993).

• Twaddell WS, Lefkowitch J, Berk PD. Evolution from primary biliary cirrhosis to primary biliary cirrhosis/autoimmune hepatitis overlap syndrome. Semin. Liver Dis. 28: 128-134 (2008).

• Ueno Y, Ishii M, Igarashi T, Mano Y, Yahagi K, Kisara N, Kobayashi Y, Niitsuma H, Kobayashi K, Shimosegawa T. Primary biliary cirrhosis with antibody against carbonic anhydrase II associates with distinct immunological backgrounds. Hepatol. Res. 20: 18-27 (2001).

• Uibo R, Mackay IR, Rowley M, Humphries P, Armstrong JM, McNeilage J. Inhibition of enzyme function by human autoantibodies to an autoantigen pyruvate dehydrogenase E2: different epitope for spontaneous human and induced rabbit autoantibodies. Clin. Exp. Immunol. 80: 19-24 (1990). *

• Uibo R, Salupere V. The epidemiology of primary biliary cirrhosis: immunological problems. Hepatogastroenterology 46: 3048-3052 (1999).

• Umar RK, Rinella ME. An update on nonalcoholic fatty liver disease. Gastroenterology & Hepatology Annual Review Vol. 1, June 2006, p. 94-98 (2006). *

• Urbanek P. Immunological aspects of alcoholic liver disease. Falk Symposium 135. Immmunological diseases of liver and gut. Prague (Czech Republic). p. 32-35. September 12 - 13 (2003). *

• Uzoegwu P, Baum H, Williamson J. Correlation of oligomycin-sensitive ATPase activity in trypanosomes with their content of an antigen to primary biliary cirrhosis. Cell Biol. Int. Rep. 8: 981-986 (1984).

• Uzoegwu PN, Baum H, Williamson J. The occurrence and localization in trypanosomes and other endo-parasites of an antigen cross-reacting with mitochondrial antibodies of primary biliary cirrhosis. Comp. Biochem. Physiol. B. 88: 1181-1189 (1987).

• Uzoegwu PN, Baum H, Williamson J. Detection of an antigen to primary biliary cirrhosis in wild type and petite mutant Saccharomyces cerevisiae. Cell Biol. Int. Rep. 8: 987-992 (1984).

• Valente EM, Abou-Sleiman PM, Caputo V, Muqit MM, Harvey K, Gispert S, Ali Z, Del Turco D, Bentivoglio AR, Healy DG, Albanese A, Nussbaum R, Gonzalez-Maldonado R et al. Hereditary early-onset Parkinson's disease caused by mutations in PINK1. Science 304: 1158-1160 (2004). *

• Valera MJM, Smok SG, Poniachik TJ, Oksenberg RD, Silva PG, Ferrario BM, Buckel GE, Brahm BJ. Primary biliary cirrhosis: a thirteen years experience. Rev. Med. Chil. 134: 469-474 (2006).

• Valesini G, Alessandri C. New facet of antiphospholipid antibodies. Ann. N. Y. Acad. Sci. 1051: 487-497 (2005). *

• Valko M, Leibfritz D, Moncol J, Cronin MT, Mazur M, Telser J. Free radicals and antioxidants in normal physiological functions and human disease. Int. J. Biochem. Cell. Biol. 39: 44-84 (2007). *

• Vallas M, Monier JC, Tourniaire J, Damon H, Girard D, Borson-Chazot F, Savoye B. Prevalence of antimitochondrial antibodies in women with Hashimoto's thyroiditis. Presse. Med. 23: 1117-1120 (1994).

• Van de Water J, Ansari A, Prindiville T, Coppel RL, Ricalton N, Kotzin BL, Liu S, Roche TE, Krams SM, Munoz S, et al. Heterogeneity of autoreactive T cell clones specific for the E2 component of the pyruvate dehydrogenase complex in primary biliary cirrhosis. J. Exp. Med. 181: 723-733 (1995). *

• Van de Water J, Ansari AA, Surh CD, Coppel R, Roche T, Bonkovsky H, Kaplan M, Gershwin ME. Evidence for the targeting by 2-oxo-dehydrogenase enzymes in the T cell response of primary biliary cirrhosis. J. Immunol. 146: 89-94 (1991).

• Van de Water J, Cooper A, Surh CD, Coppel R, Danner D, Ansari A, Dickson R, Gershwin ME. Detection of autoantibodies to recombinant mitochondrial proteins in patients with primary biliary cirrhosis. N. Engl. J. Med. 320: 1377-1380 (1989).

• Van de Water J, Fregeau D, Davis P, Ansari A, Danner D, Leung P, Coppel R, Gershwin ME. Autoantibodies of primary biliary cirrhosis recognize dihydrolipoamide acetyltransferase and inhibit enzyme function. J. Immunol. 141: 2321-2324 (1988).

• Van de Water J, Gershwin ME, Leung P, Ansari A, Coppel RL. The autoepitope of the 74-kD mitochondrial autoantigen of primary biliary cirrhosis corresponds to the functional site of dihydrolipoamide acetyltransferase. J. Exp. Med. 167: 1791-1799 (1988). *

• Van de Water J, Gerson LB, Ferrell LD, Lake JR, Coppel RL, Batts KP, Wiesner RH, Gershwin ME. Immunohistochemical evidence of disease recurrence after liver transplantation for primary biliary cirrhosis. Hepatology 24: 1079-1084 (1996). *

• Van de Water J, Shimoda S, Niho Y, Coppel R, Ansari A, Gershwin ME. The role of T cells in primary biliary cirrhosis. Semin. Liver Dis. 17: 105-113 (1997).

• Van de Water J, Turchany J, Leung PS, Lake J, Munoz S, Surh CD, Coppel R, Ansari A, Nakanuma Y, Gershwin ME. Molecular mimicry in primary biliary cirrhosis. Evidence for biliary epithelial expression of a molecule cross-reactive with pyruvate dehydrogenase complex-E2. J. Clin. Invest. 91: 2653-2664 (1993). *

• van den Bosch HM, Bunger M, de Groot PJ, van der Meijde J, Hooiveld GJ, Muller M. Gene expression of transporters and phase I/II metabolic enzymes in murine small intestine during fasting. BMC Genomics 8: 267 (2007). *

• van Deventer SJH. Cell death in the gut. Falk Symposium 140. Inflammatory Bowel Disease: Translation from Basic Research to Clinical Practice. Dubrovnik (Croatia). p. 61. May 7-8 (2004). *

• van Leeuwen DJ, Sood G, Ferrante D, Lazenby AJ, Sellers MJ. A 38-year-old African-American woman with an unusually rapid progression of "Primary Biliary Cirrhosis": a missed opportunity! Semin. Liver Dis. 22: 395-406 (2002).

• Van Norstrand MD, Malinchoc M, Lindor KD, Therneau TM, Gershwin ME, Leung PS, Dickson ER, Homburger HA. Quantitative measurement of autoantibodies to recombinant mitochondrial antigens in patients with primary biliary cirrhosis: relationship of levels of autoantibodies to disease progression. Hepatology 25: 6-11 (1997). *

• Van Pelt FN, Carey MA, Carey JB. Chapter 29. Acute and chronic liver diseases induced by drugs or xenobiotics. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 375-388 (2007). *

• Varga J, Heiman-Patterson T, Munoz S, Love LA. Myopathy with mitochondrial alterations in patients with primary biliary cirrhosis and antimitochondrial antibodies. Arthritis Rheum. 36: 1468-1475 (1993).

• Ventriglia M, Chiavetto LB, Scassellati C, Squitti R, Binetti G, Ghidoni R, Rossini PM, Gennarelli M. Lack of association between MnSOD gene polymorphism and sporadic Alzheimer's disease. Aging Clin. Exp. Res. 17: 445-448 (2005).

• Vergani D, Mieli-Vergani G. Autoimmune serology in liver disease: methodology and interpretation. J. Gastroenterol. Hepatol. 19: S287-S289 (2004). *

• Vergani D, Mieli-Vergani G. Chapter 20. Autoimmune hepatitis. In "Liver Immunology: Principles and Practice" (eds. Gershwin ME, Vierling JM, Manns MP), Humana Press Inc., Totowa, NJ, p. 263-275 (2007). *

• Verny C, Amati-Bonneau P, Letournel F, Person B, Dib N, Malinge MC, Slama A, Le Marechal C, Ferec C, Procaccio V, Reynier P, Bonneau D. Mitochondrial DNA A3243G mutation involved in familial diabetes, chronic intestinal pseudo-obstruction and recurrent pancreatitis. Diabetes Metab. 34: 620-626 (2008).

• Vick B, Weber A, Urbanik T, Maass T, Teufel A, Krammer PH, Opferman JT, Schuchmann M, Galle PR, Schulze-Bergkamen H. Knockout of myeloid cell leukemia-1 induces liver damage and increases apoptosis susceptibility of murine hepatocytes. Hepatology 49: 627-636 (2009). *

• Vidalino L, Doria A, Quarta S, Zen M, Gatta A, Pontisso P. SERPINB3, apoptosis and autoimmunity. Autoimmun. Rev. 9: 108-112 (2009).

• Vierling JM. Immune disorders of the liver and bile duct. Gastroenterol. Clin. North Am. 21: 427-449 (1992).

• Vierling JM. Primary biliary cirrhosis and autoimmune cholangiopathy. Clin. Liver Dis. 8: 177-194 (2004).

• Vilagut L, Vila J, Vinas O, Pares A, Gines A, Jimenez de Anta MT, Rodes J. Cross-reactivity of anti-Mycobacterium gordonae antibodies with the major mitochondrial autoantigens in primary biliary cirrhosis. J. Hepatol. 21: 673-677 (1994).

• Villeneuve JP, Pichette V. Cytochrome P450 and liver diseases. Curr. Drug Metab. 5: 273-282 (2004).

• Vleggaar FP, van Buuren HR. No prognostic significance of antimitochondrial antibody profile testing in primary biliary cirrhosis. Hepatogastroenterology 51: 937-940 (2004).

• Vogel A, Aslan JE, Willenbring H, Klein C, Finegold M, Mount H, Thomas G, Grompe M. Sustained phosphorylation of bid is a marker for resistance to fas-induced apoptosis during chronic liver diseases. Gastroenterology 130: 104-119 (2006). *

• von Landenberg P, Baumgartner M, Schoelmerich J, Lackner KJ, Klein R. Clinical relevance of antiphospholipid antibodies in primary biliary cirrhosis. Ann. N. Y. Acad. Sci. 1051: 20-28 (2005). *

• Voronina SG, Barrow SL, Simpson AW, Gerasimenko OV, da Silva Xavier G, Rutter GA, Petersen OH, Tepikin AV. Dynamic changes in cytosolic and mitochondrial ATP levels in pancreatic acinar cells. Gastroenterology Jan 23 [Epub ahead of print] (2010). *

• Wakabayashi K, Lian ZX, Leung PS, Moritoki Y, Tsuneyama K, Kurth MJ, Lam KS, Yoshida K, Yang GX, Hibi T, Ansari AA, Ridgway WM, Coppel RL, Mackay IR, Gershwin ME. Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease. Hepatology 48: 531-540 (2008). *

• Wakabayashi K, Lian ZX, Moritoki Y, Lan RY, Tsuneyama K, Chuang YH, Yang GX, Ridgway W, Ueno Y, Ansari AA, Coppel RL, Mackay IR, Gershwin ME. IL-2 receptor alpha(-/-) mice and the development of primary biliary cirrhosis. Hepatology 44: 1240-1249 (2006). *

• Wakabayashi K, Yoshida K, Leung PS, Moritoki Y, Yang GX, Tsuneyama K, Lian ZX, Hibi T, Ansari AA, Wicker LS, Ridgway WM, Coppel RL, Mackay IR, Gershwin ME. Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization. Clin. Exp. Immunol. 155: 577-586 (2009). *

• Wakabayashi T, Ohno H, Hayakawa Y, Kawashima A, Sawabu N. Primary biliary cirrhosis associated with type A gastritis and chronic thyroiditis. J. Gastroenterol. 34: 415-419 (1999). *

• Wang J, Jiang W, Gao H, He B, Liu W. Clinical and pathological characteristics of primary biliary cirrhosis. Zhonghua Gan Zang Bing Za Zhi 10: 334-337 (2002).

• Wang P, Liu J, Li Y, Wu S, Luo J, Yang H, Subbiah R, Chatham J, Zhelyabovska O, Yang Q. Peroxisome proliferator-activated receptor delta is an essential transcriptional regulator for mitochondrial protection and biogenesis in adult heart. Circ. Res. Jan 14 [Epub ahead of print] (2010).

• Wang S, Wang F, Shi X, Dai J, Peng Y, Guo X, Wang X, Shen H, Hu Z. Association between manganese superoxide dismutase (MnSOD) Val-9Ala polymorphism and cancer risk - a meta-analysis. Eur. J. Cancer 45: 2874-2881 (2009). *

• Wang X, Cederbaum AI. S-Adenosyl-L-methionine decreases the elevated hepatotoxicity induced by Fas agonistic antibody plus acute ethanol pretreatment in mice. Arch. Biochem. Biophys. 477: 1-11 (2008). *

• Wang XY, Zarate N, Soderholm JD, Bourgeois JM, Liu LW, Huizinga JD. Ultrastructural injury to interstitial cells of Cajal and communication with mast cells in Crohn's disease. Neurogastroenterol. Motil. 19: 349-364 (2007).

• Wang Y, Srinivasan K, Siddiqui MR, George SP, Tomar A, Khurana S. A novel role for villin in intestinal epithelial cell survival and homeostasis. J. Biol. Chem. 283: 9454-9464 (2008). *

• Warskulat U, Borsch E, Reinehr R, Heller-Stilb B, Monnighoff I, Buchczyk D, Donner M, Flogel U, Kappert G, Soboll S, Beer S, Pfeffer K, Marschall HU, Gabrielsen M, Amiry-Moghaddam M, Ottersen OP, Dienes HP, Haussinger D. Chronic liver disease is triggered by taurine transporter knockout in the mouse. FASEB J. 20: 574-576 (2006). *

• Washington K. Autoimmune cholangitis: not just AMA-negative primary biliary cirrhosis. Adv. Anat. Pathol. 9: 244-250 (2002).

• Washington MK. Autoimmune liver disease: overlap and outliers. Mod. Pathol. 20 Suppl. 1: S15-S30 (2007). *

• Wasilenko ST, Mason GE, Mason AL. Primary biliary cirrhosis, bacteria and molecular mimicry: what's the molecule and where's the mimic? Liver Int. 29: 779-782 (2009). *

• Watanabe N, Takashimizu S, Shiraishi K, Kagawa T, Nishizaki Y, Mine T, Akatsuka A, Selmi C, Gershwin ME. Primary biliary cirrhosis with multinucleated hepatocellular giant cells: implications for pathogenesis of primary biliary cirrhosis. Eur. J. Gastroenterol. Hepatol. 18: 1023-1027 (2006).

• Watanabe S, Deguchi A, Uchida N, Kurokohchi K, Arima K, Nishioka M, Hasui H. Histopathologic comparison of anti-mitochondrial antibody-positive primary biliary cirrhosis and autoimmune cholangiopathy. Hepatol. Res. 19: 41-51 (2001).

• Weber M, Lohse AW, Manns M, Meyer zum Buschenfelde KH, Kohler H. IgG subclass distribution of autoantibodies to glomerular basement membrane in Goodpasture's syndrome compared to other autoantibodies. Nephron 49: 54-57 (1988).

• Weber P, Brenner J, Stechemesser E, Klein R, Weckenmann U, Kloppel G, Kirchhof M, Fintelmann V, Berg PA. Characterization and clinical relevance of a new complement-fixing antibody - anti-M8 - in patients with primary biliary cirrhosis. Hepatology 6: 553-559 (1986).

• Weismuller TJ, Wedemeyer J, Kubicka S, Strassburg CP, Manns MP. The challenges in primary sclerosing cholangitis: aetiopathogenesis, autoimmunity, management and malignancy? J. Hepatol. 48: S38-S57 (2008). *

• Weitzel LR, Mayles WJ, Sandoval PA, Wischmeyer PE. Effects of pharmaconutrients on cellular dysfunction and the microcirculation in critical illness. Curr. Opin. Anaesthesiol. 22: 177-183 (2009).

• Welch C, Santra MK, El-Assaad W, Zhu X, Huber WE, Keys RA, Teodoro JG, Green MR. Identification of a protein, G0S2, that lacks Bcl-2 homology domains and interacts with and antagonizes Bcl-2. Cancer Res. 69: 6782-6789 (2009). *

• Wen YF, Yang JS, Kuo SC, Hwang CS, Chung JG, Wu HC, Huang WW, Jhan JH, Lin CM, Chen HJ. Investigation of anti-leukemia molecular mechanism of ITR-284, a carboxamide analog, in leukemia cells and its effects in WEHI-3 leukemia mice. Biochem. Pharmacol. 79: 389-398 (2010). *

• Wenchich L, Krechler T, Horak J, Sediva A, Bartunkova J, Hansikova H, Martasek P, Zeman J, Svestka T. Primary biliary cirrhosis - specific anti-mitochondrial antibodies. Vnitr. Lek. 50: 842-845 (2004).

• Wendel M, Heller AR. Anticancer actions of omega-3 fatty acids - current state and future perspectives. Anticancer Agents Med. Chem. 9: 457-470 (2009).

• Wenzel U, Nickel A, Kuntz S, Daniel H. Ascorbic acid suppresses drug-induced apoptosis in human colon cancer cells by scavenging mitochondrial superoxide anions. Carcinogenesis 25: 703-712 (2004). *

• Wesierska-Gadek J, Penner E, Battezzati PM, Selmi C, Zuin M, Hitchman E, Worman HJ, Gershwin ME, Podda M, Invernizzi P. Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis. Hepatology 43: 1135-1144 (2006). *

• Weston SR, Jorgensen RA, Dickson ER, Lindor KD. Is routine cholangiography useful in men with suspected primary biliary cirrhosis? J. Clin. Gastroenterol. 29: 68-70 (1999).

• Weyman RL, Voigt M. Consecutive occurrence of primary biliary cirrhosis and autoimmune hepatitis: a case report and review of the literature. Am. J. Gastroenterol. 96: 585-587 (2001). *

• Whiteman M, Chu SH, Siau JL, Rose P, Sabapathy K, Schantz JT, Cheung NS, Spencer JP, Armstrong JS. The pro-inflammatory oxidant hypochlorous acid induces Bax-dependent mitochondrial permeabilisation and cell death through AIF-/EndoG-dependent pathways. Cell. Signal. 19: 705-714 (2007). *

• Whyte J, Hough D, Maddison PJ, McHugh NJ. The association of primary biliary cirrhosis and systemic sclerosis is not accounted for by cross reactivity between mitochondrial and centromere antigens. J. Autoimmun. 7: 413-424 (1994).

• Widder JD, Fraccarollo D, Galuppo P, Hansen JM, Jones DP, Ertl G, Bauersachs J. Attenuation of angiotensin II-induced vascular dysfunction and hypertension by overexpression of thioredoxin 2. Hypertension 54: 338-344 (2009).

• Wiesner RH, LaRusso NF, Ludwig J, Dickson ER. Comparison of the clinicopathologic features of primary sclerosing cholangitis and primary biliary cirrhosis. Gastroenterology 88: 108-114 (1985).

• Wiesner RH, Ludwig J, Lindor KD, Jorgensen RA, Baldus WP, Homburger HA, Dickson ER. A controlled trial of cyclosporine in the treatment of primary biliary cirrhosis. N. Engl. J. Med. 322: 1419-1424 (1990).

• Willerson JT, Kulkarni P, Stone M, Lewis SE, Eigenbrodt E, Bonte FJ, Parkey RW, Buja LM. Localization of anti-mitochondrial antibody in experimental canine myocardial infarcts. Proc. Natl. Acad. Sci. U.S.A. 77: 6856-6859 (1980). *

• Winter SL, Kraft SC, Boyer JL. Antimitochondrial antibodies: reagent variables may lead to diagnostic error. Dig. Dis. Sci. 24: 15-20 (1979).

• Wirth HP, Meyenberger C, Ammann R, Blum HE. Parietal cell antibodies in primary biliary cirrhosis: pathogenetic or diagnostic significance? Schweiz. Med. Wochenschr. 124: 816-820 (1994).

• Wirth HP, Zala G, Meyenberger C, Ammann R. Significance of subtype pattern of antimitochondrial antibodies in primary biliary cirrhosis for prognostic parameters and response to ursodeoxycholic acid. Schweiz. Med. Wochenschr. 125: 750-754 (1995).

• Wiswedel I. F(2)-isoprostanes: sensitive biomarkers of oxidative stress in vitro and in vivo: a gas chromatography-mass spectrometric approach. Methods Mol. Biol. 580: 3-16 (2009).

• Witt-Sullivan H, Heathcote J, Cauch K, Blendis L, Ghent C, Katz A, Milner R, Pappas SC, Rankin J, Wanless IR. The demography of primary biliary cirrhosis in Ontario, Canada. Hepatology 12: 98-105 (1990).

• Wollina U, Graefe T, Oelzner P, Hein G, Schreiber G. Pseudoainhum of all fingers associated with Reynolds' syndrome and breast cancer: report of a case and review of the literature. J. Am. Acad. Dermatol. 44: 381-384 (2001).

• Wong GL, Hui AY, Wong VW, Chan FK, Sung JJ, Chan HL. A retrospective study on clinical features and prognostic factors of biopsy-proven primary biliary cirrhosis in Chinese patients. Am. J. Gastroenterol. 100: 2205-2211 (2005). *

• Wong IL, Chow LM. The role of Leishmania enriettii multidrug resistance protein 1 (LeMDR1) in mediating drug resistance is iron-dependent. Mol. Biochem. Parasitol. 150: 278-287 (2006). *

• Wong WW, Puthalakath H. Bcl-2 family proteins: the sentinels of the mitochondrial apoptosis pathway. IUBMB Life 60: 390-397 (2008). *

• Worman HJ, Courvalin JC. Antinuclear antibodies specific for primary biliary cirrhosis. Autoimmun. Rev. 2: 211-217 (2003).

• Wu CH, Xu XY, Lu HY, Yu YY, Wang GQ. Clinical and pathological analysis of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Beijing Da Xue Xue Bao 36: 609-611 (2004).

• Wu CH, Xu XY, Tian GS, Yu YY. Serum autoantibodies of patients with chronic hepatitis C and the significance thereof in infection of hepatitis C virus. Zhonghua Yi Xue Za Zhi 86: 390-393 (2006).

• Wu D, Cederbaum AI. Development and properties of HepG2 cells that constitutively express CYP2E1. Methods Mol. Biol. 447: 137-150 (2008). *

• Wu JS, Cheung WM, Tsai YS, Chen YT, Fong WH, Tsai HD, Chen YC, Liou JY, Shyue SK, Chen JJ, Chen YE, Maeda N, Wu KK, Lin TN. Ligand-activated peroxisome proliferator-activated receptor-gamma protects against ischemic cerebral infarction and neuronal apoptosis by 14-3-3varepsilon upregulation. Circulation 119: 1124-1134 (2009). *

• Wu JS, Lin TN, Wu KK. Rosiglitazone and PPAR-gamma overexpression protect mitochondrial membrane potential and prevent apoptosis by upregulating anti-apoptotic Bcl-2 family proteins. J. Cell. Physiol. 220: 58-71 (2009). *

• Wu KK, Liou JY. Cyclooxygenase inhibitors induce colon cancer cell apoptosis via PPARdelta --> 14-3-3epsilon pathway. Methods Mol. Biol. 512: 295-307 (2009).

• Wu X, Zhang L, Gurley E, Studer E, Shang J, Wang T, Wang C, Yan M, Jiang Z, Hylemon PB, Sanyal AJ, Pandak WM Jr, Zhou H. Prevention of free fatty acid-induced hepatic lipotoxicity by 18beta-glycyrrhetinic acid through lysosomal and mitochondrial pathways. Hepatology 47: 1905-1915 (2008). *

• Xerri L, Nosny Y, Minko D, Benkoel L, Nouhou H, Kohler JL, Chamlian A. Association of sarcoidosis and primary biliary cirrhosis. Clinical and anatomopathologic study of a case followed for over 10 years. Gastroenterol. Clin. Biol. 13: 513-516 (1989).

• Xia Q, Lu F, Yan HP, Wang HX, Feng X, Zhao Y, Liu BY, Wang J, Li P, Xue Y, Hu MR, Qian L, Guo N, Yang SC, Li MY, Ma YF, Li BA, Zhang XM, Shen BF. Autoantibody profiling of Chinese patients with autoimmune hepatitis using immunoproteomic analysis. J. Proteome Res. 7: 1963-1970 (2008). *

• Xiong Y, Fang JH, Yun JP, Yang J, Zhang Y, Jia WH, Zhuang SM. Effects of microRNA-29 on apoptosis, tumorigenicity, and prognosis of hepatocellular carcinoma. Hepatology Oct 19 [Epub ahead of print] (2009).

• Xu L, Shen Z, Guo L, Fodera B, Keogh A, Joplin R, O'Donnell B, Aitken J, Carman W, Neuberger J, Mason A. Does a betaretrovirus infection trigger primary biliary cirrhosis? Proc. Natl. Acad. Sci. U.S.A. 100: 8454-8459 (2003). *

• Xu Z, Lin S, Wu W, Tan H, Wang Z, Cheng C, Lu L, Zhang X. Ghrelin prevents doxorubicin-induced cardiotoxicity through TNF-alpha/NF-kappaB pathways and mitochondrial protective mechanisms. Toxicology 247: 133-138 (2008). *

• Yabu K, Kiyosawa K, Akahane Y, Shimakura K, Nagata A, Furuta S. A case of asymptomatic primary biliary cirrhosis with an initial presenting feature of localized gastric varices. Gastroenterol. Jpn. 18: 384-390 (1983).

• Yamamoto H, Watanabe T, Mizuno H, Endo K, Fukushige J, Hosokawa T, Kazusaka A, Fujita S. The antioxidant effect of DL-alpha-lipoic acid on copper-induced acute hepatitis in Long-Evans Cinnamon (LEC) rats. Free Radic. Res. 34: 69-80 (2001).

• Yamamoto K, Terada R, Okamoto R, Hiasa Y, Abe M, Onji M, Tsuji T. A scoring system for primary biliary cirrhosis and its application for variant forms of autoimmune liver disease. J. Gastroenterol. 38: 52-59 (2003). *

• Yamamoto S, Yokota T, Fukui D, Shibahara T, Ishikawa Y, Kadota K. Foci of altered hepatocytes in a fox squirrel (Sciurus niger) with haemochromatosis. J. Comp. Pathol. 131: 237-241 (2004). *

• Yamashiki M, Kosaka Y, Nishioka J, Tameda Y, Takase K, Watanabe S, Kaito M, Nishimura A, Suzuki H, Nomoto M. Flow cytometric analysis of IL-6 receptors on peripheral lymphocytes in patients with primary biliary cirrhosis. J. Clin. Lab. Anal. 12: 83-87 (1998). *

• Yanagawa T, Miyakawa H, Shibata M, Kawaguchi N, Ishibashi M, Goto N, Mitamura K. Immunoreactivity to pyruvate dehydrogenase complex-E2 in well-defined patients with autoimmune hepatitis: Western blot analysis. Hepatol. Res. 26: 81-86 (2003).

• Yang D, Elner SG, Bian ZM, Till GO, Petty HR, Elner VM. Pro-inflammatory cytokines increase reactive oxygen species through mitochondria and NADPH oxidase in cultured RPE cells. Exp. Eye Res. 85: 462-472 (2007). *

• Yang GX, Lian ZX, Chuang YH, Moritoki Y, Lan RY, Wakabayashi K, Ansari AA, Flavell RA, Ridgway WM, Coppel RL, Tsuneyama K, Mackay IR, Gershwin ME. Adoptive transfer of CD8(+) T cells from transforming growth factor beta receptor type II (dominant negative form) induces autoimmune cholangitis in mice. Hepatology 47: 1974-1982 (2008). *

• Yang RZ, Park S, Reagan WJ, Goldstein R, Zhong S, Lawton M, Rajamohan F, Qian K, Liu L, Gong DW. Alanine aminotransferase isoenzymes: molecular cloning and quantitative analysis of tissue expression in rats and serum elevation in liver toxicity. Hepatology 49: 598-607 (2009). *

• Yao D, Xie W, Wang L. Clinical analysis of 22 patients with primary biliary cirrhosis. Zhonghua Gan Zang Bing Za Zhi 10: 344-345 (2002).

• Yao DK, Xie WF, Chen WZ, Liu HY, Tu XQ, Fan LY. The value of antimitochondrial antibody and its subtypes in the diagnosis of primary biliary cirrhosis. Zhonghua Gan Zang Bing Za Zhi 13: 9-11 (2005).

• Yao J, Jiang Z, Duan W, Huang J, Zhang L, Hu L, He L, Li F, Xiao Y, Shu B, Liu C. Involvement of mitochondrial pathway in triptolide-induced cytotoxicity in human normal liver L-02 cells. Biol. Pharm. Bull. 31: 592-597 (2008). *

• Yao Y, Xu ZY, Gao JP, Chen XP, Shen LJ, Yang LH, You LY, Yang JH. Liver histopathology, clinical features and prognostic factors of primary biliary cirrhosis: an analysis of 60 cases. Zhonghua Gan Zang Bing Za Zhi 16: 457-460 (2008).

• Yap I, Wee A, Tay HH, Guan R, Kang JY. Primary biliary cirrhosis - an uncommon disease in Singapore. Singapore Med. J. 37: 48-50 (1996).

• Yasuda S, Kobayashi H, Iwasa M, Kawamura I, Sumi S, Narentuoya B, Yamaki T, Ushikoshi H, Nishigaki K, Nagashima K, Takemura G, Fujiwara T, Fujiwara H, Minatoguchi S. Anti-diabetic drug pioglitazone protects the heart via activation of PPAR-gamma, PI3 kinase, Akt and eNOS pathway in a rabbit model of myocardial infarction. Am. J. Physiol. Heart Circ. Physiol. 296: H1558-H1565 (2009). *

• Yasuda Y, Nakano S, Akiguchi I, Tanaka M, Kameyama M. Polymyositis associated with asymptomatic primary biliary cirrhosis. Eur. Neurol. 33: 51-53 (1993).

• Yasukawa K, Oshiumi H, Takeda M, Ishihara N, Yanagi Y, Seya T, Kawabata S, Koshiba T. Mitofusin 2 inhibits mitochondrial antiviral signaling. Sci. Signal. 2: ra47 (2009). *

• Yeaman SJ, Fussey SP, Danner DJ, James OF, Mutimer DJ, Bassendine MF. Primary biliary cirrhosis: identification of two major M2 mitochondrial autoantigens. Lancet 1: 1067-1070 (1988).

• Yee SW, Campbell MJ, Simons C. Inhibition of vitamin D(3) metabolism enhances VDR signalling in androgen-independent prostate cancer cells. J. Steroid Biochem. Mol. Biol. 98: 228-235 (2006).

• Yerushalmi B, Dahl R, Devereaux MW, Gumpricht E, Sokol RJ. Bile acid-induced rat hepatocyte apoptosis is inhibited by antioxidants and blockers of the mitochondrial permeability transition. Hepatology 33: 616-626 (2001). *

• Yip TT, Van de Water J, Gershwin ME, Coppel RL, Hutchens TW. Cryptic antigenic determinants on the extracellular pyruvate dehydrogenase complex/mimeotope found in primary biliary cirrhosis. A probe by affinity mass spectrometry. J. Biol. Chem. 271: 32825-32833 (1996). *

• Yoon JH, Gores GJ. Diagnosis, staging, and treatment of cholangiocarcinoma. Curr. Treat. Options Gastroenterol. 6: 105-112 (2003).

• Yoshida EM, Singh RA, Vartanian RK, Owen DA, Erb SR, Scudamore CH. Late recurrent post-transplant primary biliary cirrhosis in British Columbia. Can. J. Gastroenterol. 11: 229-233 (1997). *

• Yoshida K, Yang GX, Zhang W, Tsuda M, Tsuneyama K, Moritoki Y, Ansari AA, Okazaki K, Lian ZX, Coppel RL, Mackay IR, Gershwin ME. Deletion of interleukin-12p40 suppresses autoimmune cholangitis in dominant negative transforming growth factor beta receptor type II mice. Hepatology 50: 1494-1500 (2009). *

• Yoshida T, Bonkovsky H, Ansari A, Danner D. Antibodies against mitochondrial dehydrogenase complexes in primary biliary cirrhosis. Gastroenterology 99: 187-194 (1990).

• Yoshizawa K, Shirakawa H, Ichijo T, Umemura T, Tanaka E, Kiyosawa K, Imagawa E, Matsuda K, Hidaka E, Sano K, Nakazawa Y, Ikegami T, Hashikura Y, Miyagawa S, Ota M, Nakano M. De novo autoimmune hepatitis following living-donor liver transplantation for primary biliary cirrhosis. Clin. Transplant. 22: 385-390 (2008). *

• You X, Liu W, Zhang X, Zhang FC. Patterns of anti-nuclear antibodies in patients with primary biliary cirrhosis and primary Sjogren syndrome. Zhonghua Yi Xue Za Zhi 88: 168-170 (2008).

• Yousefi S, Gold JA, Andina N, Lee JJ, Kelly AM, Kozlowski E, Schmid I, Straumann A, Reichenbach J, Gleich GJ, Simon HU. Catapult-like release of mitochondrial DNA by eosinophils contributes to antibacterial defense. Nat. Med. 14: 949-953 (2008). *

• Yu LC, Flynn AN, Turner JR, Buret AG. SGLT-1-mediated glucose uptake protects intestinal epithelial cells against LPS-induced apoptosis and barrier defects: a novel cellular rescue mechanism? FASEB J. 19: 1822-1835 (2005). *

• Yuan L, Kaplowitz N. Glutathione in liver diseases and hepatotoxicity. Mol. Aspects Med. 30: 29-41 (2009).

• Zachou K, Rigopoulou E, Liaskos C, Patsiaoura K, Makri E, Stathakis N, Dalekos GN. Primary biliary cirrhosis presented as peripheral eosinophilia in asymptomatic women with or without elevated alkaline phosphatase. Eur. J. Gastroenterol. Hepatol. 16: 425-428 (2004).

• Zamora M, Merono C, Vinas O, Mampel T. Recruitment of NF-kappa B into mitochondria is involved in ANT1 (adenine nucleotide translocase 1) induced apoptosis. J. Biol. Chem. 279: 38415-38423 (2004). *

• Zanotto-Filho A, Schroder R, Moreira JC. Differential effects of retinol and retinoic acid on cell proliferation: a role for reactive species and redox-dependent mechanisms in retinol supplementation. Free Radic. Res. 42: 778-788 (2008).

• Zein CO, Angulo P, Lindor KD. When is liver biopsy needed in the diagnosis of primary biliary cirrhosis? Clin. Gastroenterol. Hepatol. 1: 89-95 (2003). *

• Zeng Z, Han YK, Zhang XF, Geng H. Clinical features of 35 cases of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome. Zhonghua Gan Zang Bing Za Zhi 13: 3-5 (2005).

• Zephir H, Almeras L, El Behi M, Dussart P, de Seze J, Steibel J, Trifilieff E, Dubucquoi S, Dessaint JP, Vermersch P, Prin L, Lefranc D. Diversified serum IgG response involving non-myelin CNS proteins during experimental autoimmune encephalomyelitis. J. Neuroimmunol. 179: 53-64 (2006).

• Zhang A, Liu Y, Shen Y, Xu Y, Li X. Osteopontin silencing by small interfering RNA induces apoptosis and suppresses invasion in human renal carcinoma Caki-1 cells. Med. Oncol. Nov 17 [Epub ahead of print] (2009). *

• Zhang D, Zhou Z, Li L, Weng J, Huang G, Jing P, Zhang C, Peng J, Xiu L. Islet autoimmunity and genetic mutations in Chinese subjects initially thought to have type 1B diabetes. Diabet. Med. 23: 67-71 (2006). *

• Zhang F, Jia J, Cui R, Wang B, Wang H. Clinical features of forty patients with primary biliary cirrhosis. Chin. Med. J. (Engl.) 115: 904-908 (2002).

• Zhang F, Jia J, Wang B, Qian L, Yin S, Wang Y, Cui Y, You H, Ma H, Wang H, Zhang C. Clinical characteristics of primary biliary cirrhosis: a report of 45 cases. Zhonghua Nei Ke Za Zhi 41: 163-167 (2002).

• Zhang FK, Jia JD, Wang BE. Clinical evaluation of serum antimitochondrial antibody-negative primary biliary cirrhosis. Hepatobiliary Pancreat. Dis. Int. 3: 288-291 (2004). *

• Zhang L, Jayne DR, Oliveira DB. Anti-idiotype antibodies to anti-mitochondrial antibodies in the sera of patients with primary biliary cirrhosis. J. Autoimmun. 6: 93-105 (1993). *

• Zhang L, Weetman AP, Jayne DR, Turner I, Yeaman SJ, Bassendine MF, Oliveira DB. Anti-mitochondrial antibody IgG subclass distribution and affinity in primary biliary cirrhosis. Clin. Exp. Immunol. 88: 56-61 (1992). *

• Zhang LY, Lu YP, Yang L, Luo GH, Song J, Li YP. Detection of citrate synthase autoantibodies in rats with chronic allograft nephropathy. Transplant. Proc. 41: 4366-4368 (2009). *

• Zhang W, Moritoki Y, Tsuneyama K, Yang GX, Ilan Y, Lian ZX, Gershwin ME. Beta-glucosylceramide ameliorates liver inflammation in murine autoimmune cholangitis. Clin. Exp. Immunol. 157: 359-364 (2009). *

• Zhang W, Sharma R, Ju ST, He XS, Tao Y, Tsuneyama K, Tian Z, Lian ZX, Fu SM, Gershwin ME. Deficiency in regulatory T cells results in development of antimitochondrial antibodies and autoimmune cholangitis. Hepatology 49: 545-552 (2009). *

• Zhang Y, Ba Y, Liu C, Sun G, Ding L, Gao S, Hao J, Yu Z, Zhang J, Zen K, Tong Z, Xiang Y, Zhang CY. PGC-1alpha induces apoptosis in human epithelial ovarian cancer cells through a PPARgamma-dependent pathway. Cell Res. 17: 363-373 (2007). *

• Zhang Y, Yao S, Li J. Vegetable-derived isothiocyanates: anti-proliferative activity and mechanism of action. Proc. Nutr. Soc. 65: 68-75 (2006). *

• Zhao J, Dong L, Lu B, Wu G, Xu D, Chen J, Li K, Tong X, Dai J, Yao S, Wu M, Guo Y. Down-regulation of osteopontin suppresses growth and metastasis of hepatocellular carcinoma via induction of apoptosis. Gastroenterology 135: 956-968 (2008). *

• Zhao P, Sharma AC, Ren J. Pathogenesis and therapy of autoimmunity-induced dilated cardiomyopathy. Front. Biosci. 14: 1708-1715 (2009).

• Zhao Y, Ding WX, Qian T, Watkins S, Lemasters JJ, Yin XM. Bid activates multiple mitochondrial apoptotic mechanisms in primary hepatocytes after death receptor engagement. Gastroenterology 125: 854-867 (2003). *

• Zhong B, Strnad P, Selmi C, Invernizzi P, Tao GZ, Caleffi A, Chen M, Bianchi I, Podda M, Pietrangelo A, Gershwin ME, Omary MB. Keratin variants are over-represented in primary biliary cirrhosis and associate with disease severity. Hepatology 50: 546-554 (2009). *

• Zhong B, Zhang L, Lei C, Li Y, Mao AP, Yang Y, Wang YY, Zhang XL, Shu HB. The ubiquitin ligase RNF5 regulates antiviral responses by mediating degradation of the adaptor protein MITA. Immunity 30: 397-407 (2009). *

• Zhou D, Zaiger G, Ghebremeskel K, Crawford MA, Reifen R. Vitamin A deficiency reduces liver and colon docosahexaenoic acid levels in rats fed high linoleic and low alpha-linolenic acid diet. Prostaglandins Leukot. Essent. Fatty Acids 71: 383-389 (2004). *

• Zimmermann K, Cueni B, Schmid M. Combined form of chronic aggressive hepatitis primary biliary cirrhosis. Schweiz. Med. Wochenschr. 107: 1749-1752 (1977).

• Zuber MA, Recktenwald C. Clinical correlation of antimitochondrial antibodies. Eur. J. Med. Res. 8: 61-70 (2003).

• Zurgil N, Bakimer R, Kaplan M, Youinou P, Shoenfeld Y. Anti-pyruvate dehydrogenase autoantibodies in primary biliary cirrhosis. J. Clin. Immunol. 11: 239-245 (1991).

• Zurgil N, Bakimer R, Moutsopoulos HM, Tzioufas AG, Youinou P, Isenberg DA, Scheinberg M, Kveder T, Rozman B, Luderschmidt C, et al. Antimitochondrial (pyruvate dehydrogenase) autoantibodies in autoimmune rheumatic diseases. J. Clin. Immunol. 12: 201-209 (1992).

• Zwingmann C, Bilodeau M. Metabolic insights into the hepatoprotective role of N-acetylcysteine in mouse liver. Hepatology 43: 454-463 (2006). *

Number of references = 1509

www psc-literature.org