• Accatino L, Pizarro M, Solis N, Arrese M, Vollrath V, Ananthanarayanan M, Chianale J, Koenig CS 2000 Differential expression of canalicular membrane Ca2+/Mg(2+)-ecto-ATPase in estrogen-induced and obstructive cholestasis in the rat. J. Lab. Clin. Med. 136: 125-137. *

• Adams DH, Afford SC 2005 Effector mechanisms of nonsuppurative destructive cholangitis in graft-versus-host disease and allograft rejection. Semin. Liver Dis. 25: 281-297.

• Adams DH, Afford SC 2002 The role of cholangiocytes in the development of chronic inflammatory liver disease. Front. Biosci. 7: 276-285.

• Adams DH, Afford SC, Lalor PF 2003 Cellular immunoreactions in the development 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. 27. October 15-16. *

• Alabraba EB, Lai V, Boon L, Wigmore SJ, Adams DH, Afford SC 2008 Coculture of human liver macrophages and cholangiocytes leads to CD40-dependent apoptosis and cytokine secretion. Hepatology 47: 552-562.

• Alpini G, Baiocchi L, Glaser S, Ueno Y, Marzioni M, Francis H, Phinizy JL, Angelico M, Lesage G 2002 Ursodeoxycholate and tauroursodeoxycholate inhibit cholangiocyte growth and secretion of BDL rats through activation of PKC alpha. Hepatology 35: 1041-1052. *

• Alpini G, Glaser S, Baiocchi L, Francis H, Xia X, Lesage G 2005 Secretin activation of the apical Na(+)-dependent bile acid transporter is associated with cholehepatic shunting in rats. Hepatology 41: 1037-1045. *

• Alpini G, Glaser SS, Ueno Y, Rodgers R, Phinizy JL, Francis H, Baiocchi L, Holcomb LA, Caligiuri A, LeSage GD 1999 Bile acid feeding induces cholangiocyte proliferation and secretion: evidence for bile acid-regulated ductal secretion. Gastroenterology 116: 179-186. *

• Alpini G, McGill JM, LaRusso NF 2002 The pathobiology of biliary epithelia. Hepatology 35: 1256-1268. *

• Alvaro D, Gigliozzi A, Attili AF 2000 Regulation and deregulation of cholangiocyte proliferation. J. Hepatol. 33: 333-340. *

• Alvaro D, Invernizzi P, Onori P, Franchitto A, De Santis A, Crosignani A, Sferra R, Ginanni-Corradini S, Mancino MG, Maggioni M, Attili AF, Podda M, Gaudio E 2004 Estrogen receptors in cholangiocytes and the progression of primary biliary cirrhosis. J. Hepatol. 41: 905-912. *

• Alvaro D, Mancino MG, Glaser S, Gaudio E, Marzioni M, Francis H, Alpini G 2007 Proliferating cholangiocytes: a neuroendocrine compartment in the diseased liver. Gastroenterology 132: 415-431. *

• Alvaro D, Mancino MG, Onori P, Franchitto A, Alpini G, Francis H, Glaser S, Gaudio E 2006 Estrogens and the pathophysiology of the biliary tree. World J. Gastroenterol. 12: 3537-3545. *

• Alvaro D, Metalli VD, Alpini G, Onori P, Franchitto A, Barbaro B, Glaser SS, Francis H, Cantafora A, Blotta I, Attili AF, Gaudio E 2005 The intrahepatic biliary epithelium is a target of the growth hormone/insulin-like growth factor 1 axis. J. Hepatol. 43: 875-883. *

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

• Arenas F, Hervias I, Uriz M, Joplin R, Prieto J, Medina JF 2008 Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells. J. Clin. Invest. 118: 695-709. *

• Arrese M, Accatino L 2002 From blood to bile: recent advances in hepatobiliary transport. Ann. Hepatol. 1: 64-71. *

• Baghdasaryan A, Fickert P, Fuchsbichler A, Silbert D, Gumhold J, Horl G, Langner C, Moustafa T, Halilbasic E, Claudel T, Trauner M 2008 Role of hepatic phospholipids in development of liver injury in Mdr2 (Abcb4) knockout mice. Liver Int. 28: 948-958. *

• Baiocchi L, LeSage G, Glaser S, Alpini G 1999 Regulation of cholangiocyte bile secretion. J. Hepatol. 31: 179-191. *

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

• Balistreri WF 1997 Bile acid therapy in pediatric hepatobiliary disease: the role of ursodeoxycholic acid. J. Pediatr. Gastroenterol. Nutr. 24: 573-589.

• Ballatori N, Christian WV, Lee JY, Dawson PA, Soroka CJ, Boyer JL, Madejczyk MS, Li N 2005 OSTalpha-OSTbeta: a major basolateral bile acid and steroid transporter in human intestinal, renal, and biliary epithelia. Hepatology 42: 1270-1279. *

• Banales JM, Arenas F, Rodriguez-Ortigosa CM, Saez E, Uriarte I, Doctor RB, Prieto J, Medina JF 2006 Bicarbonate-rich choleresis induced by secretin in normal rat is taurocholate-dependent and involves AE2 anion exchanger. Hepatology 43: 266-275. *

• Banales JM, Prieto J, Medina JF 2006 Cholangiocyte anion exchange and biliary bicarbonate excretion. World J. Gastroenterol. 12: 3496-3511. *

• Beaussier M, Wendum D, Fouassier L, Rey C, Barbu V, Lasnier E, Lienhart A, Scoazec JY, Rosmorduc O, Housset C 2005 Adaptative bile duct proliferative response in experimental bile duct ischemia. J. Hepatol. 42: 257-265. *

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

• Beuers U 2006 Drug insight: mechanisms and sites of action of ursodeoxycholic acid in cholestasis. Nat. Clin. Pract. Gastroenterol. Hepatol. 3: 318-328. *

• Beuers U 2006 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. *

• Beuers U 2003 Mechanisms of action of ursodeoxycholic acid in cholestasis. 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. 49-50. October 15-16. *

• Bihl F, Emmenegger U, Reichen J, Neftel KA, Zimmermann A, Cerny A 2006 Macrophage activating syndrome is associated with lobular hepatitis and severe bile duct injury with cholestasis. J. Hepatol. 44: 1208-1212. *

• Bijvelds MJ, Jorna H, Verkade HJ, Bot AG, Hofmann F, Agellon LB, Sinaasappel M, de Jonge HR 2005 Activation of CFTR by ASBT-mediated bile salt absorption. Am. J. Physiol. Gastrointest. Liver Physiol. 289: G870-G879. *

• Bogert PT, LaRusso NF 2007 Cholangiocyte biology. Curr. Opin. Gastroenterol. 23: 299-305.

• Bolder U, Trang NV, Hagey LR, Schteingart CD, Ton-Nu HT, Cerre C, Elferink RP, Hofmann AF 1999 Sulindac is excreted into bile by a canalicular bile salt pump and undergoes a cholehepatic circulation in rats. Gastroenterology 117: 962-971. *

• Boucherie S, Koukoui O, Nicolas V, Combettes L 2005 Cholestatic bile acids inhibit gap junction permeability in rat hepatocyte couplets and normal rat cholangiocytes. J. Hepatol. 42: 244-251 (2005). *

• Boyer JL, Denk G, Bohan A, Chen WS, Soroka C, Denson L 2004 Adaptive regulation of bile salt transporters in cholestasis. Falk Symposium 141. XVIII International Bile Acid Meeting: Bile Acid Biology and its Therapeutic Implications. Stockholm (Sweden). p. 46-47. June 18-19. *

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

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

• Byrne JA, Meara NJ, Rayner AC, Thompson RJ, Knisely AS 2007 Lack of hepatocellular CD10 along bile canaliculi is physiologic in early childhood and persistent in Alagille syndrome. Lab. Invest. 87: 1138-1148. *

• Chazouilleres O 2004 Primary sclerosing cholangitis and biliary malignancy: a glimmer of hope? J. Hepatol. 40: 857-859. *

• Chen XM, Levine SA, Tietz P, Krueger E, McNiven MA, Jefferson DM, Mahle M, LaRusso NF 1998 Cryptosporidium parvum is cytopathic for cultured human biliary epithelia via an apoptotic mechanism. Hepatology 28: 906-913. *

• Chiba T, Kita K, Zheng YW, Yokosuka O, Saisho H, Iwama A, Nakauchi H, Taniguchi H 2006 Side population purified from hepatocellular carcinoma cells harbors cancer stem cell-like properties. Hepatology 44: 240-251. *

• Cho WK, Boyer JL 1999 Vasoactive intestinal polypeptide is a potent regulator of bile secretion from rat cholangiocytes. Gastroenterology 117: 420-428. *

• Cho WK, Siegrist VJ, Zinzow W 2004 Impaired regulatory volume decrease in freshly isolated cholangiocytes from cystic fibrosis mice: implications for cystic fibrosis transmembrane conductance regulator effect on potassium conductance. J. Biol. Chem. 279: 14610-14618. *

• Colombo C 2006 Liver disease in cystic fibrosis. Falk Symposium 156. XIII Falk Liver Week (Part II). GENETICS IN LIVER DISEASES, Freiburg (Germany), October 8-9, 2006, p. 31-33. *

• Colombo C, Battezzati PM, Strazzabosco M, Podda M 1998 Liver and biliary problems in cystic fibrosis. Semin. Liver Dis. 18: 227-235.

• Cramer T, Schuppan D, Bauer M, Pfander D, Neuhaus P, Herbst H 2004 Hepatocyte growth factor and c-Met expression in rat and human liver fibrosis. Liver Int. 24: 335-344. *

• Demeilliers C, Jacquemin E, Barbu V, Mergey M, Paye F, Fouassier L, Chignard N, Housset C, Lomri NE 2006 Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation. Hepatology 43: 1125-1134. *

• Demetris AJ, Specht S, Nozaki I, Lunz JG 3rd, Stolz DB, Murase N, Wu T 2008 Small proline-rich proteins (SPRR) function as SH3 domain ligands, increase resistance to injury and are associated with epithelial-mesenchymal transition (EMT) in cholangiocytes. J. Hepatol. 48: 276–288. *

• Demorrow S, Francis H, Gaudio E, Ueno Y, Venter J, Onori P, Franchitto A, Vaculin B, Vaculin S, Alpini G 2008 Anandamide inhibits cholangiocyte hyperplastic proliferation via activation of thioredoxin 1/redox factor 1 and AP-1 activation. Am. J. Physiol. Gastrointest. Liver Physiol. 294: G506-G519. *

• Derkow K, Loddenkemper C, Mintern J, Kruse N, Klugewitz K, Berg T, Wiedenmann B, Ploegh HL, Schott E 2007 Differential priming of CD8 and CD4 T-cells in animal models of autoimmune hepatitis and cholangitis. Hepatology 46: 1155-1165.

• Desmet VJ 2003 Histology of autoimmune cholestatic liver diseases. 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. 25-26. October 15-16. *

• Diwakar V, Pearson L, Beath S 2001 Liver disease in children with cystic fibrosis. Paediatr. Respir. Rev. 2: 340-349.

• Doctor RB, Fouassier L 2002 Emerging roles of the actin cytoskeleton in cholangiocyte function and disease. Semin. Liver Dis. 22: 263-276.

• Donner MG, Keppler D 2001 Up-regulation of basolateral multidrug resistance protein 3 (Mrp3) in cholestatic rat liver. Hepatology 34: 351-359. *

• Dutta AK, Woo K, Doctor RB, Fitz JG, Feranchak AP 2008 Extracellular nucleotides stimulate Cl- currents in biliary epithelia through receptor-mediated IP3 and Ca2+ release. Am. J. Physiol. Gastrointest. Liver Physiol. Sep 11 [Epub ahead of print]. *

• Eksteen B, Afford SC, Wigmore SJ, Holt AP, Adams DH 2007 Immune-mediated liver injury. Semin. Liver Dis. 27: 351-366.

• Eksteen B, Miles A, Curbishley SM, Tselepis C, Grant AJ, Walker LS, Adams DH 2006 Epithelial inflammation is associated with CCL28 production and the recruitment of regulatory T cells expressing CCR10. J. Immunol. 177: 593-603. *

• Elias E 2005 Hepatology highlights. Hepatology 42: 1241-1242. *

• Elsing C, Kassner A, Stremmel W 1996 Sodium, hydrogen antiporter activation by extracellular adenosine triphosphate in biliary epithelial cells. Gastroenterology 111: 1321-1332. *

• Fabris L, Cadamuro M, Fiorotto R, Roskams T, Spirli C, Melero S, Sonzogni A, Joplin RE, Okolicsanyi L, Strazzabosco M 2006 Effects of angiogenic factor overexpression by human and rodent cholangiocytes in polycystic liver diseases. Hepatology 43: 1001-1012. *

• Fallon MB 2005 Mechanisms of pulmonary vascular complications of liver disease: hepatopulmonary syndrome. J. Clin. Gastroenterol. 39: S138-S142.

• Fava G, Glaser S, Francis H, Alpini G 2005 The immunophysiology of biliary epithelium. Semin. Liver Dis. 25: 251-264.

• Fava G, Marzioni M, Francis H, Glaser S, Demorrrow S, Ueno Y, Benedetti A, Alpini G 2007 Novel interaction of bile acid and neural signaling in the regulation of cholangiocyte function. Hepatol. Res. 37 Suppl. 3: S420-S429. *

• Fava G, Ueno Y, Glaser S, Francis H, Demorrow S, Marucci L, Marzioni M, Benedetti A, Venter J, Vaculin B, Vaculin S, Alpini G 2007 Thyroid hormone inhibits biliary growth in bile duct ligated rats by PLC/IP3/Ca2+-dependent downregulation of SRC/ERK1/2. Am. J. Physiol. Cell Physiol. 292: C1467-C1475. *

• Feranchak AP 2004 Hepatobiliary complications of cystic fibrosis. Curr. Gastroenterol. Rep. 6: 231-239.

• Feranchak AP, Doctor RB, Troetsch M, Brookman K, Johnson SM, Fitz JG 2004 Calcium-dependent regulation of secretion in biliary epithelial cells: the role of apamin-sensitive SK channels. Gastroenterology 127: 903-913. *

• Feranchak AP, Fitz JG 2002 Adenosine triphosphate release and purinergic regulation of cholangiocyte transport. Semin. Liver Dis. 22: 251-262.

• Feranchak AP, Sokol RJ 2001 Cholangiocyte biology and cystic fibrosis liver disease. Semin. Liver Dis. 21: 471-488.

• Festi D, Montagnani M, Azzaroli F, Lodato F, Mazzella G, Roda A, Di Biase AR, Roda E, Simoni P, Colecchia A 2007 Clinical efficacy and effectiveness of ursodeoxycholic acid in cholestatic liver diseases. Curr. Clin. Pharmacol. 2: 155-177.

• Fickert P, Fuchsbichler A, Moustafa T, Halilbasic E, Denk H, Trauner M 2006 Bile acids and liver fibrosis - causative agent and therapeutic tool. 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. 70-71. *

• Fickert P, Stoger U, Fuchsbichler A, Moustafa T, Marschall HU, Weiglein AH, Tsybrovskyy O, Jaeschke H, Zatloukal K, Denk H, Trauner M 2007 A new xenobiotic-induced mouse model of sclerosing cholangitis and biliary fibrosis. Am. J. Pathol. 171: 525-536. *

• Fickert P, Wagner M, Marschall HU, Fuchsbichler A, Zollner G, Tsybrovskyy O, Zatloukal K, Liu J, Waalkes MP, Cover C, Denk H, Hofmann AF, Jaeschke H, Trauner M 2006 24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice. Gastroenterology 130: 465-481. *

• Fiorotto R, Spirli C, Fabris L, Cadamuro M, Okolicsanyi L, Strazzabosco M 2007 Ursodeoxycholic acid stimulates cholangiocyte fluid secretion in mice via CFTR-dependent ATP secretion. Gastroenterology 133: 1603-1613. *

• Fitz JG 2002 Regulation of cholangiocyte secretion. Semin. Liver Dis. 22: 241-249.

• Fitz JG 2002 Current concepts in developmental, physiologic, and pathophysiologic aspects of cholangiocyte biology. Semin. Liver Dis. 22: 211-212.

• Fouassier L, Beaussier M, Schiffer E, Rey C, Barbu V, Mergey M, Wendum D, Callard P, Scoazec JY, Lasnier E, Stieger B, Lienhart A, Housset C 2007 Hypoxia-induced changes in the expression of rat hepatobiliary transporter genes. Am. J. Physiol. Gastrointest. Liver Physiol. 293: G25-G35. *

• Francis H, Glaser S, Ueno Y, LeSage G, Marucci L, Benedetti A, Taffetani S, Marzioni M, Alvaro D, Venter J, Reichenbach R, Fava G, Phinizy JL, Alpini G 2004 cAMP stimulates the secretory and proliferative capacity of the rat intrahepatic biliary epithelium through changes in the PKA/Src/MEK/ERK1/2 pathway. J. Hepatol. 41: 528-537. *

• Frei P, Gao B, Hagenbuch B, Mate A, Biber J, Murer H, Meier PJ, Stieger B 2005 Identification and localization of sodium/phosphate cotransporters in hepatocytes and cholangiocytes of rat liver. Am. J. Physiol. Gastrointest. Liver Physiol. 288: G771-G778. *

• Freudenberg F, Broderick AL, Yu BB, Leonard MR, Glickman JN, Carey MC 2008 Pathophysiologic basis of liver disease in cystic fibrosis employing a DeltaF508 mouse model. Am. J. Physiol. Gastrointest. Liver Physiol. 294: G1411-G1420. *

• Fukushima K, Ueno Y 2006 Bioinformatic approach for understanding the heterogeneity of cholangiocytes. World J. Gastroenterol. 12: 3481-3486. *

• Gatto M, Drudi-Metalli V, Torrice A, Alpini G, Cantafora A, Blotta I, Alvaro D 2008 Insulin-like growth factor-1 isoforms in rat hepatocytes and cholangiocytes and their involvement in protection against cholestatic injury. Lab. Invest. 88: 986-994. *

• Gaudio E, Barbaro B, Alvaro D, Glaser S, Francis H, Ueno Y, Meininger CJ, Franchitto A, Onori P, Marzioni M, Taffetani S, Fava G, Stoica G, Venter J, Reichenbach R, De Morrow S, Summers R, Alpini G 2006 Vascular endothelial growth factor stimulates rat cholangiocyte proliferation via an autocrine mechanism. Gastroenterology 130: 1270-1282. *

• Gaudio E, Franchitto A, Pannarale L, Carpino G, Alpini G, Francis H, Glaser S, Alvaro D, Onori P 2006 Cholangiocytes and blood supply. World J. Gastroenterol. 12: 3546-3552. *

• Gaudio E, Onori P, Franchitto A, Pannarale L, Alpini G, Alvaro D 2005 Hepatic microcirculation and cholangiocyte physiopathology. Ital. J. Anat. Embryol. 110: 71S-75S.

• Geier A, Dietrich CG, Lammert F, Orth T, Mayet WJ, Matern S, Gartung C 2002 Regulation of organic anion transporters in a new rat model of acute and chronic cholangitis resembling human primary sclerosing cholangitis. J. Hepatol. 36: 718-724. *

• Gershwin ME, Van de Water J 2001 Cholangiocytes and primary biliary cirrhosis: prediction and predication. J. Clin. Invest. 108: 187-188. *

• Gigliozzi A, Alpini G, Svegliati Baroni G, Marucci L, Drudi Metalli V, Glaser SS, Francis H, Grazia Mancino M, Ueno Y, Barbaro B, Benedetti A, Attili AF, Alvaro D 2004 Nerve growth factor modulates the proliferative capacity of the intrahepatic biliary epithelium in experimental cholestasis. Gastroenterology 127: 1198-1209. *

• Glaser S, Francis H, Demorrow S, Lesage G, Fava G, Marzioni M, Venter J, Alpini G 2006 Heterogeneity of the intrahepatic biliary epithelium. World J. Gastroenterol. 12: 3523-3536. *

• Glaser SS, Demorrow S, Francis H, Ueno Y, Gaudio E, Vaculin S, Venter J, Franchitto A, Onori P, Vaculin B, Marzioni M, Wise CE, Pilanthananond M, Savage J, Pierce L, Mancinelli R, Alpini G 2008 Progesterone stimulates the proliferation of female and male cholangiocytes via autocrine/paracrine mechanisms. Am. J. Physiol. Gastrointest. Liver Physiol. 295: G124-G136. *

• Glaser SS, Ueno Y, Demorrow S, Chiasson VL, Katki KA, Venter J, Francis HL, Dickerson IM, Dipette DJ, Supowit SC, Alpini GD 2007 Knockout of alpha-calcitonin gene-related peptide reduces cholangiocyte proliferation in bile duct ligated mice. Lab. Invest. 87: 914-926. *

• Gradilone SA, Masyuk AI, Splinter PL, Banales JM, Huang BQ, Tietz PS, Masyuk TV, Larusso NF 2007 Cholangiocyte cilia express TRPV4 and detect changes in luminal tonicity inducing bicarbonate secretion. Proc. Natl. Acad. Sci. U.S.A. 104: 19138-19143. *

• Greenbaum LE 2008 Hedgehog signaling in biliary fibrosis. J. Clin. Invest. Sep 18 [Epub ahead of print]. *

• Gressner OA, Weiskirchen R, Gressner AM 2007 Evolving concepts of liver fibrogenesis provide new diagnostic and therapeutic options. Comp. Hepatol. 6: 7. *

• Guettier C 2005 Which stem cells for adult liver? Ann. Pathol. 25: 33-44.

• Hadj-Rabia S, Baala L, Vabres P, Hamel-Teillac D, Jacquemin E, Fabre M, Lyonnet S, De Prost Y, Munnich A, Hadchouel M, Smahi A 2004 Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease. Gastroenterology 127: 1386-1390. *

• Halilbasic E, Fickert P, Foirotto R , Marschall HU, Moustafa T, Fuchsbichler A, Gumhold J, Silbert D, Langner C, Maitra U, Denk H , Strazzabosco M, Trauner M 2008 Side-chain-modification critically determines the physiologic and therapeutic properties of 24-nor-ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice and isolated bile duct units. Falk Symposium 165: XX International Bile Acid Meeting. Bile Acid Biology and Therapeutic Actions. June 13-14, 2008, Amsterdam, Netherlands, p. 55. *

• Hanada S, Harada M, Koga H, Kawaguchi T, Taniguchi E, Kumashiro R, Ueno T, Ueno Y, Ishii M, Sakisaka S, Sata M 2003 Tumor necrosis factor-alpha and interferon-gamma directly impair epithelial barrier function in cultured mouse cholangiocytes. Liver Int. 23: 3-11. *

• He Y, Wu GD, Sadahiro T, Noh SI, Wang H, Talavera D, Wang H, Vierling JM, Klein AS 2008 Interaction of CD44 and hyaluronic acid enhances biliary epithelial proliferation in cholestatic livers. Am. J. Physiol. Gastrointest. Liver Physiol. 295: G305-G312. *

• Hirata K, Nathanson MH 2001 Bile duct epithelia regulate biliary bicarbonate excretion in normal rat liver. Gastroenterology 121: 396-406. *

• Hirohashi T, Suzuki H, Takikawa H, Sugiyama Y 2000 ATP-dependent transport of bile salts by rat multidrug resistance-associated protein 3 (Mrp3). J. Biol. Chem. 275: 2905-2910. *

• Hofmann AF 1998 Progress in idiopathic bile acid malabsorption. Gut 43: 738-739. *

• Hruz P, Zimmermann C, Gutmann H, Degen L, Beuers U, Terracciano L, Drewe J, Beglinger C 2006 Adaptive regulation of the ileal apical sodium dependent bile acid transporter (ASBT) in patients with obstructive cholestasis. Gut 55: 395-402. *

• Hu M, Kurobe M, Jeong YJ, Fuerer C, Ghole S, Nusse R, Sylvester KG 2007 Wnt/beta-catenin signaling in murine hepatic transit amplifying progenitor cells. Gastroenterology 133: 1579-1591. *

• Hu W, Blazar BR, Manivel JC, Paradis K, Sharp HL 1996 Phenotypical and functional characterization of intrahepatic bile duct cells from common duct ligated mice. J. Lab. Clin. Med. 128: 536-544.

• Hulzebos CV, Voshol PJ, Wolters H, Kruit JK, Ottenhof R, Groen AK, Stellaard F, Verkade HJ, Kuipers F 2005 Bile duct proliferation associated with bile salt-induced hypercholeresis in Mdr2 P-glycoprotein-deficient mice. Liver Int. 25: 604-612. *

• Ishimura N, Bronk SF, Gores GJ 2005 Inducible nitric oxide synthase up-regulates Notch-1 in mouse cholangiocytes: implications for carcinogenesis. Gastroenterology 128: 1354-1368. *

• Ishimura N, Bronk SF, Gores GJ 2004 Inducible nitric oxide synthase upregulates cyclooxygenase-2 in mouse cholangiocytes promoting cell growth. Am. J. Physiol. Gastrointest. Liver Physiol. 287: G88-G95. *

• Ishimura N, Isomoto H, Bronk SF, Gores GJ 2006 TRAIL induces cell migration and invasion in apoptosis-resistant cholangiocarcinoma cells. Am. J. Physiol. Gastrointest. Liver Physiol. 290: G129-G136. *

• Isomoto H, Kobayashi S, Werneburg NW, Bronk SF, Guicciardi ME, Frank DA, Gores GJ 2005 Interleukin 6 upregulates myeloid cell leukemia-1 expression through a STAT3 pathway in cholangiocarcinoma cells. Hepatology 42: 1329-1338. *

• Jaeckel E, Manns MP 2003 The role of autoantigens and autoantibodies in PBC. 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. 28. October 15-16. *

• Jafri M, Donnelly B, Allen S, Bondoc A, McNeal M, Rennert PD, Weinreb PH, Ward R, Tiao G 2008 Cholangiocyte expression of alpha2beta1 integrin confers susceptibility to rotavirus induced experimental biliary atresia. Am. J. Physiol. Gastrointest. Liver Physiol. 295: G16-G26. *

• Jaiswal M, LaRusso NF, Shapiro RA, Billiar TR, Gores GJ 2001 Nitric oxide-mediated inhibition of DNA repair potentiates oxidative DNA damage in cholangiocytes. Gastroenterology 120: 190-199. *

• Jansen PL 2006 Genetics of cholestasis. Falk Symposium 156. XIII Falk Liver Week (Part II). GENETICS IN LIVER DISEASES, Freiburg (Germany), October 8-9, 2006, p. 37-40. *

• Jansen PL 2000 The pathophysiology of cholestasis with special reference to primary biliary cirrhosis. Baillieres Best Pract. Res. Clin. Gastroenterol. 14: 571-583.

• Jung D, Fried M, Kullak-Ublick GA 2002 Human apical sodium-dependent bile salt transporter gene (SLC10A2) is regulated by the peroxisome proliferator-activated receptor alpha. J. Biol. Chem. 277: 30559-30566. *

• Kamiya A, Kakinuma S, Onodera M, Miyajima A, Nakauchi H 2008 Prospero-related homeobox 1 and liver receptor homolog 1 coordinately regulate long-term proliferation of murine fetal hepatoblasts. Hepatology 48: 252-264.

• Kanno N, LeSage G, Glaser S, Alpini G 2001 Regulation of cholangiocyte bicarbonate secretion. Am. J. Physiol. Gastrointest. Liver Physiol. 281: G612-G625. *

• Karlsen TH, Boberg KM, Schrumpf E 2006 Role of polymorphisms in genes involved in bile acid detoxification pathways in primary sclerosing cholangitis. 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. 87. *

• Karpen SJ, Crawford JM 1999 Cellular and molecular biology of the liver. Curr. Opin. Gastroenterol. 15: 184-191.

• Katoonizadeh A, Nevens F, Verslype C, Pirenne J, Roskams T 2006 Liver regeneration in acute severe liver impairment: a clinicopathological correlation study. Liver Int. 26: 1225-1233. *

• Khan SA, Taylor-Robinson SD, Carmichael PL, Habib N, Lemoine NR, Thomas HC 2006 Analysis of p53 mutations for a mutational signature in human intrahepatic cholangiocarcinoma. Int. J. Oncol. 28: 1269-1277. *

• Kip NS, Lazaridis KN, Masyuk AI, Splinter PL, Huebert RC, LaRusso NF 2004 Differential expression of cholangiocyte and ileal bile acid transporters following bile acid supplementation and depletion. World J. Gastroenterol. 10: 1440-1446. *

• Kodali P, Wu P, Lahiji PA, Brown EJ, Maher JJ 2006 ANIT toxicity toward mouse hepatocytes in vivo is mediated primarily by neutrophils via CD18. Am. J. Physiol. Gastrointest. Liver Physiol. 291: G355-G363. *

• Kruglov EA, Jain D, Dranoff JA 2002 Isolation of primary rat liver fibroblasts. J. Investig. Med. 50: 179-184.

• Kullak-Ublick GA, Meier PJ 2000 Mechanisms of cholestasis. Clin. Liver Dis. 4: 357-385.

• Kullak-Ublick GA, Stieger B, Meier PJ 2004 Enterohepatic bile salt transporters in normal physiology and liver disease. Gastroenterology 126: 322-342. *

• Lai GH, Zhang Z, Shen XN, Ward DJ, Dewitt JL, Holt SE, Rozich RA, Hixson DC, Sirica AE 2005 erbB-2/neu transformed rat cholangiocytes recapitulate key cellular and molecular features of human bile duct cancer. Gastroenterology 129: 2047-2057. *

• Lammert F, Marschall HU, Matern S 2003 Intrahepatic cholestasis of pregnancy. Curr. Treat. Options Gastroenterol. 6: 123-132.

• Lazaridis KN, LaRusso NF 2003 Bile formation: do not ignore the role of plasma membrane-cytoskeleton linking proteins. Hepatology 37: 218-220. *

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

• Lazaridis KN, Pham L, Tietz P, Marinelli RA, deGroen PC, Levine S, Dawson PA,LaRusso NF 1997 Rat cholangiocytes absorb bile acids at their apical domain via the ileal sodium-dependent bile acid transporter. J. Clin. Invest. 100: 2714-2721. *

• Lazaridis KN, Tietz P, Wu T, Kip S, Dawson PA, LaRusso NF 2000 Alternative splicing of the rat sodium/bile acid transporter changes its cellular localization and transport properties. Proc. Natl. Acad. Sci. U.S.A. 97: 11092-11097. *

• Lee FY, Kast-Woelbern HR, Chang J, Luo G, Jones SA, Fishbein MC, Edwards PA 2005 alpha-Crystallin is a target gene of the farnesoid X-activated receptor in human livers. J. Biol. Chem. 280: 31792-31800. *

• Lee J, Azzaroli F, Wang L, Soroka CJ, Gigliozzi A, Setchell KD, Kramer W, Boyer JL 2001 Adaptive regulation of bile salt transporters in kidney and liver in obstructive cholestasis in the rat. Gastroenterology 121: 1473-1484. *

• Lee J, Boyer JL 2000 Molecular alterations in hepatocyte transport mechanisms in acquired cholestatic liver disorders. Semin. Liver Dis. 20: 373-384. *

• Lee SO 2008 Physiologic and pathologic experimental models for studying cholangiocytes. Korean J. Hepatol. 14: 139-149.

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

• LeSage GD, Alvaro D, Glaser S, Francis H, Marucci L, Roskams T, Phinizy JL, Marzioni M, Benedetti A, Taffetani S, Barbaro B, Fava G, Ueno Y, Alpini G 2004 Alpha-1 adrenergic receptor agonists modulate ductal secretion of BDL rats via Ca(2+)- and PKC-dependent stimulation of cAMP. Hepatology 40: 1116-1127. *

• LeSage GD, Benedetti A, Glaser S, Marucci L, Tretjak Z, Caligiuri A, Rodgers R, Phinizy JL, Baiocchi L, Francis H, Lasater J, Ugili L, Alpini G 1999 Acute carbon tetrachloride feeding selectively damages large, but not small, cholangiocytes from normal rat liver. Hepatology 29: 307-319. *

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

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

• Li L, Falany CN 2007 Elevated hepatic SULT1E1 activity in mouse models of cystic fibrosis alters the regulation of estrogen responsive proteins. J. Cyst. Fibros. 6: 23-30.

• Lie-A-Ling M, Bakker CT, Deurholt T, Hoekstra R, Wesseling JG, Afford SC, Bosma PJ 2006 Selection of tumour specific promoters for adenoviral gene therapy of cholangiocarcinoma. J. Hepatol. 44: 126-133. *

• Limaye PB, Alarcon G, Walls AL, Nalesnik MA, Michalopoulos GK, Demetris AJ, Ochoa ER 2008 Expression of specific hepatocyte and cholangiocyte transcription factors in human liver disease and embryonic development. Lab. Invest. 88: 865-872. *

• Malhi H, Gores GJ 2006 Review article: the modern diagnosis and therapy of cholangiocarcinoma. Aliment. Pharmacol. Ther. 23: 1287-1296. *

• Malhi H, Gores GJ 2006 Cholangiocarcinoma: modern advances in understanding a deadly old disease. J. Hepatol. 45: 856-867. *

• Mancino MG, Carpino G, Onori P, Franchitto A, Alvaro D, Gaudio E 2007 Hepatic "stem" cells: state of the art. Ital. J. Anat. Embryol. 112: 93-109.

• Marienfeld C, Yamagiwa Y, Ueno Y, Chiasson V, Brooks L, Meng F, Patel T 2004 Translational regulation of XIAP expression and cell survival during hypoxia in human cholangiocarcinoma. Gastroenterology 127: 1787-1797. *

• Marinelli RA, Gradilone SA, Carreras FI, Calamita G, Lehmann GL 2004 Liver aquaporins: significance in canalicular and ductal bile formation. Ann. Hepatol. 3: 130-136. *

• Marinelli RA, Tietz PS, LaRusso NF 2005 Regulated vesicle trafficking of membrane transporters in hepatic epithelia. J. Hepatol. 42: 592-603. *

• Marra F, Defranco R, Robino G, Novo E, Efsen E, Pastacaldi S, Zamara E, Vercelli A, Lottini B, Spirli C, Strazzabosco M, Pinzani M, Parola M 2005 Thiazolidinedione treatment inhibits bile duct proliferation and fibrosis in a rat model of chronic cholestasis. World J. Gastroenterol. 11: 4931-4938. *

• Martin J, Dufour JF 2004 Cholestasis shuts down calcium signaling in cholangiocytes. Hepatology 39: 248-249. *

• Marucci L, Ugili L, Macarri G, Feliciangeli G, Bendia E, Jezequel AM, Orlandi F, Benedetti A 2001 Primary biliary cirrhosis: modalities of injury and death in biliary epithelium. Dig. Liver Dis. 33: 576-583.

• Marzioni M, Alpini G, Saccomanno S, Candelaresi C, Venter J, Rychlicki C, Fava G, Francis H, Trozzi L, Glaser S, Benedetti A 2007 Glucagon-like peptide-1 and its receptor agonist exendin-4 modulate cholangiocyte adaptive response to cholestasis. Gastroenterology 133: 244-255. *

• Marzioni M, Alpini G, Saccomanno S, De Minicis S, Glaser S, Francis H, Trozzi L, Venter J, Orlando F, Fava G, Candelaresi C, Macarri G, Benedetti A 2006 Endogenous opioids modulate the growth of the biliary tree in the course of cholestasis. Gastroenterology 130: 1831-1847. *

• Marzioni M, Fava G, Alvaro D, Alpini G, Benedetti A 2008 Control of cholangiocyte adaptive responses by visceral hormones and neuropeptides. Clin. Rev. Allergy Immunol. Jun 11 [Epub ahead of print].

• Marzioni M, Fava G, Benedetti A 2006 Nervous and neuroendocrine regulation of the pathophysiology of cholestasis and of biliary carcinogenesis. World J. Gastroenterol. 12: 3471-3480. *

• Marzioni M, Francis H, Benedetti A, Ueno Y, Fava G, Venter J, Reichenbach R, Mancino MG, Summers R, Alpini G, Glaser S 2006 Ca2+-dependent cytoprotective effects of ursodeoxycholic and tauroursodeoxycholic acid on the biliary epithelium in a rat model of cholestasis and loss of bile ducts. Am. J. Pathol. 168: 398-409. *

• Marzioni M, Glaser S, Francis H, Marucci L, Benedetti A, Alvaro D, Taffetani S, Ueno Y, Roskams T, Phinizy JL, Venter J, Fava G, Lesage GD, Alpini G 2005 Autocrine/paracrine regulation of the growth of the biliary tree by the neuroendocrine hormone serotonin. Gastroenterology 128: 121-137. *

• Marzioni M, Glaser S, Ueno Y, Francis H, Venter J, Alvaro D, Benedetti A, Fava G, Mancino MG, Alpini G, LeSage G 2006 Ca2+-dependent cytoprotective effects of ursodeoxycholic and taurocholic acids on bile duct injury induced by damage of sympathetic and parasympathetic innervation. 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. 41-42. *

• Marzioni M, Glaser SS, Francis H, Phinizy JL, LeSage G, Alpini G 2002 Functional heterogeneity of cholangiocytes. Semin. Liver Dis. 22: 227-240.

• Marzioni M, Ueno Y, Glaser S, Francis H, Benedetti A, Alvaro D, Venter J, Fava G, Alpini G 2007 Cytoprotective effects of taurocholic acid feeding on the biliary tree after adrenergic denervation of the liver. Liver Int. 27: 558-568. *

• Masyuk AI, Masyuk TV, Splinter PL, Huang BQ, Stroope AJ, LaRusso NF 2006 Cholangiocyte cilia detect changes in luminal fluid flow and transmit them into intracellular ca(2+) and cAMP signaling. Gastroenterology 131: 911-920. *

• Masyuk TV, Masyuk AI, Torres VE, Harris PC, LaRusso NF 2007 Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate. Gastroenterology 132: 1104-1116. *

• Mato TK, Davis PA, Odin JA, Coppel RL, Gershwin ME 2004 Sidechain biology and the immunogenicity of PDC-E2, the major autoantigen of primary biliary cirrhosis. Hepatology 40: 1241-1248. *

• Medina J, Sanz-Cameno P, Garcia-Buey L, Martin-Vilchez S, Lopez-Cabrera M, Moreno-Otero R 2005 Evidence of angiogenesis in primary biliary cirrhosis: an immunohistochemical descriptive study. J. Hepatol. 42: 124-131. *

• Melero S, Spirli C, Zsembery A, Medina JF, Joplin RE, Duner E, Zuin M, Neuberger JM, Prieto J, Strazzabosco M 2002 Defective regulation of cholangiocyte Cl-/HCO3- and Na+/H+ exchanger activities in primary biliary cirrhosis. Hepatology 35: 1513-1521. *

• Mendez-Sanchez N, Zamora-Valdes D, Pichardo-Bahena R, Barredo-Prieto B, Ponciano-Rodriguez G, Bermejo-Martinez L, Chavez-Tapia NC, Baptista-Gonzalez HA, Uribe M 2007 Endocannabinoid receptor CB2 in nonalcoholic fatty liver disease. Liver Int. 27: 215-219. *

• Meng F, Yamagiwa Y, Ueno Y, Patel T 2006 Over-expression of interleukin-6 enhances cell survival and transformed cell growth in human malignant cholangiocytes. J. Hepatol. 44: 1055-1065. *

• Mennone A, Biemesderfer D, Negoianu D, Yang CL, Abbiati T, Schultheis PJ, Shull GE, Aronson PS, Boyer JL 2001 Role of sodium/hydrogen exchanger isoform NHE3 in fluid secretion and absorption in mouse and rat cholangiocytes. Am. J. Physiol. Gastrointest. Liver Physiol. 280: G247-G254. *

• Mikula M, Fuchs E, Huber H, Beug H, Schulte-Hermann R, Mikulits W 2004 Immortalized p19ARF null hepatocytes restore liver injury and generate hepatic progenitors after transplantation. Hepatology 39: 628-634. *

• Milkiewicz P, Mills CO, Hubscher SG, Cardenas R, Cardenas T, Williams A, Elias E 2001 Visualization of the transport of primary and secondary bile acids across liver tissue in rats: in vivo study with fluorescent bile acids. J. Hepatol. 34: 4-10. *

• Minagawa N, Ehrlich BE, Nathanson MH 2006 Calcium signaling in cholangiocytes. World J. Gastroenterol. 12: 3466-3470. *

• Minagawa N, Nagata J, Shibao K, Masyuk AI, Gomes DA, Rodrigues MA, Lesage G, Akiba Y, Kaunitz JD, Ehrlich BE, LaRusso NF, Nathanson MH 2007 Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile. Gastroenterology 133: 1592-1602. *

• Moritoki Y, Ueno Y, Kanno N, Yamagiwa Y, Fukushima K, Gershwin ME, Shimosegawa T 2007 Amniotic epithelial cell-derived cholangiocytes in experimental cholestatic ductal hyperplasia. Hepatol. Res. 37: 286-294. *

• Moritoki Y, Ueno Y, Kanno N, Yamagiwa Y, Fukushima K, Gershwin ME, Shimosegawa T 2006 Lack of evidence that bone marrow cells contribute to cholangiocyte repopulation during experimental cholestatic ductal hyperplasia. Liver Int. 26: 457-466. *

• Moser M, Matthiesen S, Kirfel J, Schorle H, Bergmann C, Senderek J, Rudnik-Schoneborn S, Zerres K, Buettner R 2005 A mouse model for cystic biliary dysgenesis in autosomal recessive polycystic kidney disease (ARPKD). Hepatology 41: 1113-1121. *

• Nakanuma Y, Sasaki M, Ikeda H, Sato Y, Zen Y, Kosaka K, Harada K 2008 Pathology of peripheral intrahepatic cholangiocarcinoma with reference to tumorigenesis. Hepatol. Res. 38: 325-334. *

• Nakauchi H 2004 Isolation and clonal characterization of hematopoietic and liver stem cells. Cornea 23: S2-S7.

• Nelson JB, O'Hara SP, Small AJ, Tietz PS, Choudhury AK, Pagano RE, Chen XM, LaRusso NF 2006 Cryptosporidium parvum infects human cholangiocytes via sphingolipid-enriched membrane microdomains. Cell. Microbiol. 8: 1932-1945. *

• Nowak G, Ericzon BG, Nava S, Jaksch M, Westgren M, Sumitran-Holgersson S 2005 Identification of expandable human hepatic progenitors which differentiate into mature hepatic cells in vivo. Gut 54: 972-979. *

• O'Mahony CA, Vierling JM 2006 Etiopathogenesis of primary sclerosing cholangitis. Semin. Liver Dis. 26: 3-21. *

• Odin JA, Huebert RC, Casciola-Rosen L, LaRusso NF, Rosen A 2001 Bcl-2-dependent oxidation of pyruvate dehydrogenase-E2, a primary biliary cirrhosis autoantigen, during apoptosis. J. Clin. Invest. 108: 223-232. *

• Omenetti A, Porrello A, Jung Y, Yang L, Popov Y, Choi SS, Witek RP, Alpini G, Venter J, Vandongen HM, Syn WK, Baroni GS, Benedetti A, Schuppan D, Diehl AM 2008 Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans. J. Clin. Invest. Sep 18 [Epub ahead of print]. *

• Onori P, Alvaro D, Floreani A, Mancino M, Franchitto A, Guido M, Carpino G, De Santis A, Angelico M, Attili AF, Gaudio E 2007 Activation of IGF1 system characterizes cholangiocyte survival during the progression of primary biliary cirrhosis (PBC). J. Histochem. Cytochem. 55: 327-334. *

• Onori P, Franchitto A, Alvaro D, Gaudio E 2001 Immunohistochemical features of bile duct epithelial cells in normal and experimental liver conditions. Ital. J. Anat. Embryol. 106 (2 Suppl. 1): 371-378.

• Patsenker E, Popov Y, Stickel F, Jonczyk A, Goodman SL, Schuppan D 2008 Inhibition of integrin alphavbeta6 on cholangiocytes blocks transforming growth factor-beta activation and retards biliary fibrosis progression. Gastroenterology 135: 660-670. *

• Pauli-Magnus C, Stieger B, Meier Y, Kullak-Ublick GA, Meier PJ 2005 Enterohepatic transport of bile salts and genetics of cholestasis. J. Hepatol. 43: 342-357. *

• Paumgartner G 2006 Medical treatment of cholestatic liver diseases: from pathobiology to pharmacological targets. World J. Gastroenterol. 12: 4445-4451. *

• Paumgartner G, Beuers U 2002 Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology 36: 525-531. *

• Poupon R 2003 Molecular mechanisms of bile formation and cholestatic diseases. Bull. Acad. Natl. Med. 187: 1261-1274.

• Poupon R 2006 Primary biliary cirrhosis: from ursodeoxycholic acid towards targeting strategies for therapy. 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. 63. *

• Poupon R, Chignard N, Rosmorduc O, Barbu V, Housset C 2004 Biliary function and its regulation. Med. Sci. (Paris) 20: 1096-1099.

• Poupon R, Poupon RE 2004 Retrovirus infection as a trigger for primary biliary cirrhosis? Lancet 363: 260-261. *

• Prall RT, LaRusso NF 1999 Biliary tract physiology. Curr. Opin. Gastroenterol. 15: 423.

• Prall RT, LaRusso NF 2000 Biliary tract physiology. Curr. Opin. Gastroenterol. 16: 432-436.

• Pratt DS 2005 Cholestasis and cholestatic syndromes. Curr. Opin. Gastroenterol. 21: 270-274.

• Pusl T, Beuers U 2006 Ursodeoxycholic acid treatment of vanishing bile duct syndromes. World J. Gastroenterol. 12: 3487-3495. *

• Que FG, Gores GJ, LaRusso NF 1997 Development and initial application of an in vitro model of apoptosis in rodent cholangiocytes. Am. J. Physiol. 272: G106-G115.

• Reddy A, Prince M, James OF, Jain S, Bassendine MF 2004 Tamoxifen: a novel treatment for primary biliary cirrhosis? Liver Int. 24: 194-197. *

• Reichel C, Meier-Abt PJ 1997 Cholestatic liver diseases. Ther. Umsch. 54: 639-644.

• Roman RM, Feranchak AP, Salter KD, Wang Y, Fitz JG 1999 Endogenous ATP release regulates Cl- secretion in cultured human and rat biliary epithelial cells. Am. J. Physiol. 276: G1391-G1400. *

• Ros JE, Libbrecht L, Geuken M, Jansen PL, Roskams TA 2003 High expression of MDR1, MRP1, and MRP3 in the hepatic progenitor cell compartment and hepatocytes in severe human liver disease. J. Pathol. 200: 553-560. *

• Ros JE, Roskams TA, Geuken M, Havinga R, Splinter PL, Petersen BE, LaRusso NF, van der Kolk DM, Kuipers F, Faber KN, Muller M, Jansen PL 2003 ATP binding cassette transporter gene expression in rat liver progenitor cells. Gut 52: 1060-1067. *

• Roskams T 2006 Liver stem cells and their implication in hepatocellular and cholangiocarcinoma. Oncogene 25: 3818-3822. *

• Rountree CB, Wang X, Ge S, Barsky L, Zhu J, Gonzales I, Crooks GM 2007 Bone marrow fails to differentiate into liver epithelium during murine development and regeneration. Hepatology 45: 1250-1260. *

• Saji Y, Tamura S, Yoshida Y, Kiso S, Iizuka AS, Matsumoto H, Kawasaki T, Kamada Y, Matsuzawa Y, Shinomura Y 2004 Basic fibroblast growth factor promotes the trans-differentiation of mouse bone marrow cells into hepatic lineage cells via multiple liver-enriched transcription factors. J. Hepatol. 41: 545-550. *

• Salas JT, Banales JM, Sarvide S, Recalde S, Ferrer A, Uriarte I, Oude Elferink RP, Prieto J, Medina JF 2008 Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. Gastroenterology 134: 1482-1493. *

• Sanchez-Munoz D, Castellano-Megias VM, Romero-Gomez M 2007 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.

• Sandhu BS, Luketic VA 2006 Management of primary sclerosing cholangitis. Gastroenterology & Hepatology 2: 843-849. *

• Saxena R, Hytiroglou P, Thung SN, Theise ND 2002 Destruction of canals of Hering in primary biliary cirrhosis. Hum. Pathol. 33: 983-988.

• Saxena R, Theise N 2004 Canals of Hering: recent insights and current knowledge. Semin. Liver Dis. 24: 43-48.

• Scheffer GL, Kool M, de Haas M, de Vree JM, Pijnenborg AC, Bosman DK, Elferink RP, van der Valk P, Borst P, Scheper RJ 2002 Tissue distribution and induction of human multidrug resistant protein 3. Lab. Invest. 82: 193-201.

• Selmi C, Invernizzi P, Zuin M, Podda M, Seldin MF, Gershwin ME 2005 Genes and (auto)immunity in primary biliary cirrhosis. Genes Immun. 6: 543-556. *

• Sheth P, Delos Santos N, Seth A, LaRusso NF, Rao RK 2007 Lipopolysaccharide disrupts tight junctions in cholangiocyte monolayers by a c-Src-, TLR4-, and LBP-dependent mechanism. Am. J. Physiol. Gastrointest. Liver Physiol. 293: G308-G318. *

• Shibao K, Hirata K, Robert ME, Nathanson MH 2003 Loss of inositol 1,4,5-trisphosphate receptors from bile duct epithelia is a common event in cholestasis. Gastroenterology 125: 1175-1187. *

• Shneider BL 2001 Intestinal bile acid transport: biology, physiology, and pathophysiology. J. Pediatr. Gastroenterol. Nutr. 32: 407-417. *

• Shneider BL 2006 Regulation of intestinal bile acid transport in health and disease. 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. 44. *

• Shoda J, Kano M, Oda K, Kamiya J, Nimura Y, Suzuki H, Sugiyama Y, Miyazaki H, Todoroki T, Stengelin S, Kramer W, Matsuzaki Y, Tanaka N 2001 The expression levels of plasma membrane transporters in the cholestatic liver of patients undergoing biliary drainage and their association with the impairment of biliary secretory function. Am. J. Gastroenterol. 96: 3368-3378. *

• Singh SK, Mennone A, Gigliozzi A, Fraioli F, Boyer JL 2001 Cl(-)-dependent secretory mechanisms in isolated rat bile duct epithelial units. Am. J. Physiol. Gastrointest. Liver Physiol. 281: G438-G446. *

• Sinha J, Chen F, Miloh T, Burns RC, Yu Z, Shneider BL 2008 beta-Klotho and FGF 15/19 inhibit the apical sodium dependent bile acid transporter in enterocytes and cholangiocytes. Am. J. Physiol. Gastrointest. Liver Physiol. Sep 4 [Epub ahead of print]. *

• Sirica AE, Zhang Z, Lai GH, Asano T, Shen XN, Ward DJ, Mahatme A, Dewitt JL 2008 A novel "patient-like" model of cholangiocarcinoma progression based on bile duct inoculation of tumorigenic rat cholangiocyte cell lines. Hepatology 47: 1178-1190. *

• Sola S, Amaral JD, Aranha MM, Steer CJ, Rodrigues CM 2006 Modulation of hepatocyte apoptosis: cross-talk between bile acids and nuclear steroid receptors. Curr. Med. Chem. 13: 3039-3051.

• Soroka CJ, Lee JM, Azzaroli F, Boyer JL 2001 Cellular localization and up-regulation of multidrug resistance-associated protein 3 in hepatocytes and cholangiocytes during obstructive cholestasis in rat liver. Hepatology 33: 783-791. *

• Soroka CJ, Xu S, Cai SY, Boyer JL 2006 Insights into the regulation of expression of human OSTalpha-OSTbeta. 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. 32. *

• Spirli C, Fabris L, Duner E, Fiorotto R, Ballardini G, Roskams T, LaRusso NF, Sonzogni A, Okolicsanyi L, Strazzabosco M 2003 Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes. Gastroenterology 124: 737-753. *

• Spirli C, Fiorotto R, Song L, Santos-Sacchi J, Okolicsanyi L, Masier S, Rocchi L, Pia Vairetti M, De Bernard M, Melero S, Pozzan T, Strazzabosco M 2005 Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism. Gastroenterology 129: 220-233. *

• Spirli C, Granato A, Zsembery K, Anglani F, Okolicsanyi L, LaRusso NF, Crepaldi G, Strazzabosco M 1998 Functional polarity of Na+/H+ and Cl-/HCO3- exchangers in a rat cholangiocyte cell line. Am. J. Physiol. 275: G1236-G1245. *

• Spirli C, Nathanson MH, Fiorotto R, Duner E, Denson LA, Sanz JM, Di Virgilio F, Okolicsanyi L, Casagrande F, Strazzabosco M 2001 Proinflammatory cytokines inhibit secretion in rat bile duct epithelium. Gastroenterology 121: 156-169. *

• Splinter PL, Masyuk AI, Marinelli RA, LaRusso NF 2004 AQP4 transfected into mouse cholangiocytes promotes water transport in biliary epithelia. Hepatology 39: 109-116. *

• Strazzabosco M 1997 Transport systems in cholangiocytes: their role in bile formation and cholestasis. Yale J. Biol. Med. 70: 427-434.

• Strazzabosco M, Fabris L, Spirli C 2005 Pathophysiology of cholangiopathies. J. Clin. Gastroenterol. 39: S90-S102.

• Strazzabosco M, Joplin R, Zsembery A, Wallace L, Spirli C, Fabris L, Granato A, Rossanese A, Poci C, Neuberger JM, Okolicsanyi L, Crepaldi G 1997 Na(+)-dependent and -independent Cl-/HCO3- exchange mediate cellular HCO3- transport in cultured human intrahepatic bile duct cells. Hepatology 25: 976-985. *

• Strazzabosco M, Spirli C, Okolicsanyi L 2000 Pathophysiology of the intrahepatic biliary epithelium. J. Gastroenterol. Hepatol. 15: 244-253. *

• Taffetani S, Glaser S, Francis H, Demorrow S, Ueno Y, Alvaro D, Marucci L, Marzioni M, Fava G, Venter J, Vaculin S, Vaculin B, Lam IP, Lee VH, Gaudio E, Carpino G, Benedetti A, Alpini G 2007 Prolactin stimulates the proliferation of normal female cholangiocytes by differential regulation of Ca2+-dependent PKC isoforms. BMC Physiol. 7: 6. *

• Takeda K, Kojima Y, Ikejima K, Harada K, Yamashina S, Okumura K, Aoyama T, Frese S, Ikeda H, Haynes NM, Cretney E, Yagita H, Sueyoshi N, Sato N, Nakanuma Y, Smyth MJ, Okumura K 2008 Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease. Proc. Natl. Acad. Sci. U.S.A. 105: 10895-10900. *

• Tamai K, Fukushima K, Ueno Y, Moritoki Y, Yamagiwa Y, Kanno N, Jefferson DM, Shimosegawa T 2006 Differential expressions of aquaporin proteins in human cholestatic liver diseases. Hepatol. Res. 34: 99-103.

• Tanaka S, Yamamoto T, Tanaka H, Kodai S, Ogawa M, Ichikawa T, Hai S, Sakabe K, Uenishi T, Shuto T, Kubo S 2005 Potentiality of combined hepatocellular and intrahepatic cholangiocellular carcinoma originating from a hepatic precursor cell: immunohistochemical evidence. Hepatol. Res. 32: 52-57.

• Taniai M, Higuchi H, Burgart LJ, Gores GJ 2002 p16INK4a promoter mutations are frequent in primary sclerosing cholangitis (PSC) and PSC-associated cholangiocarcinoma. Gastroenterology 123: 1090-1098. *

• Theise ND 2003 Hepatocytes from extrahepatic stem cells. Falk Symposium 138. XII FALK LIVER WEEK 2003 (Part III) In Honour of Hans Popper’s 100th Birthday. State-of-the-art of hepatology: Molecular and Cell Biology. Freiburg (Germany). p. 34. October 20 - 21. *

• Theise ND, Nimmakayalu M, Gardner R, Illei PB, Morgan G, Teperman L, Henegariu O, Krause DS 2000 Liver from bone marrow in humans. Hepatology 32: 11-16. *

• Thompson R, Jansen PL 2000 Genetic defects in hepatocanalicular transport. Semin. Liver Dis. 20: 365-372.

• Thorgeirsson SS, Grisham JW 2003 Overview of recent experimental studies on liver stem cells. Semin. Liver Dis. 23: 303-312.

• Tietz P, LaRusso NF 2002 Cholangiocyte biology. Curr. Opin. Gastroenterol. 18: 360-365.

• Tietz PS, LaRusso NF 2005 Cholangiocyte biology. Curr. Opin. Gastroenterol. 21: 337-343.

• Tietz PS, LaRusso NF 2004 Cholangiocyte biology. Curr. Opin. Gastroenterol. 20: 270-274.

• Tietz PS, LaRusso NF 2006 Cholangiocyte biology. Curr. Opin. Gastroenterol. 22: 279-287.

• Tietz PS, Marinelli RA, Chen XM, Huang B, Cohn J, Kole J, McNiven MA, Alper S, LaRusso NF 2003 Agonist-induced coordinated trafficking of functionally related transport proteins for water and ions in cholangiocytes. J. Biol. Chem. 278: 20413-20419. *

• Trauner M, Boyer JL 2004 Cholestatic syndromes. Curr. Opin. Gastroenterol. 20: 220-230.

• Trauner M, Boyer JL 2003 Bile salt transporters: molecular characterization, function, and regulation. Physiol. Rev. 83: 633-671. *

• Trauner M, Fickert P, Moustafa T, Guelly C, Fuchsbichler A, Denk H, Wagner M, Zollner G 2006 Side chain-shortened bile acids for the treatment of cholestasis: lessons from nor-ursodeoxycholic acid. 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. 77-78. *

• Trauner M, Fickert P, Stauber RE 2000 Hepatocellular bile salt transport: lessons from cholestasis. Can. J. Gastroenterol. 14 Suppl. D: 99D-104D.

• Tsuboi K, Tazuma S, Nishioka T, Chayama K 2004 Partial characterization of cytoprotective mechanisms of lecithin against bile salt-induced bile duct damage. J. Gastroenterol. 39: 955-960. *

• Ueno Y, Francis H, Glaser S, Demorrow S, Venter J, Benedetti A, Fava G, Marzioni M, Alpini G 2007 Taurocholic acid feeding prevents tumor necrosis factor-alpha-induced damage of cholangiocytes by a PI3K-mediated pathway. Exp. Biol. Med. (Maywood) 232: 942-949.

• Ueno Y, Fukushima K, Nakagome Y, Kido O, Shimosegawa T 2007 Bioinformatic approach for cholangiocyte pathophysiology. Hepatol. Res. 37 Suppl. 3: S444-S448. *

• Ueno Y, Ishii M, Takahashi S, Igarashi T, Toyota T, LaRusso NF 1998 Different susceptibility of mice to immune-mediated cholangitis induced by immunization with carbonic anhydrase II. Lab. Invest. 78: 629-637.

• Ueno Y, Moritoki Y, Shimosegawa T, Gershwin ME 2007 Primary biliary cirrhosis: what we know and what we want to know about human PBC and spontaneous PBC mouse models. J. Gastroenterol. 42: 189-195. *

• Ueno Y, Phillips JO, Ludwig J, Lichtman SN, LaRusso NF 1996 Development and characterization of a rodent model of immune-mediated cholangitis. Proc. Natl. Acad. Sci. U.S.A. 93: 216-220. *

• Vierling JM 2004 Primary sclerosing cholangitis: is autoimmunity involved in pathogenesis? Falk Symposium 142. GASTROENTEROLOGY WEEK FREIBURG 2004 (Part I) AUTOIMMUNE LIVER DISEASE, Freiburg (Germany), pp. 43-44. October 12-13. *

• Wang H, Vohra BP, Zhang Y, Heuckeroth RO 2005 Transcriptional profiling after bile duct ligation identifies PAI-1 as a contributor to cholestatic injury in mice. Hepatology 42: 1099-1108. *

• Williams KT, Young SP, Negus A, Young LS, Adams DH, Afford SC 2007 C4b binding protein binds to CD154 preventing CD40 mediated cholangiocyte apoptosis: a novel link between complement and epithelial cell survival. PLoS ONE 2: e159. *

• Woo K, Dutta AK, Patel V, Kresge C, Feranchak AP 2008 Fluid-flow induces mechanosensitive ATP release, calcium signalling, and Cl- transport in biliary epithelial cells through a PKCzeta-dependent pathway. J. Physiol. 586: 2779-2798. *

• Xia X, Demorrow S, Francis H, Glaser S, Alpini G, Marzioni M, Fava G, Lesage G 2007 Cholangiocyte injury and ductopenic syndromes. Semin. Liver Dis. 27: 401-412.

• Xia X, Francis H, Glaser S, Alpini G, Lesage G 2006 Bile acid interactions with cholangiocytes. World J. Gastroenterol. 12: 3553-3563. *

• Xia X, Roundtree M, Merikhi A, Lu X, Shentu S, LeSage G 2004 Degradation of the apical sodium-dependent bile acid transporter (ASBT) by the Ubiquitin-proteasome pathway in cholangiocytes. J. Biol. Chem. 279: 44931-44937. *

• Yamazaki K, Gleich GJ, Kita H 2001 Bile acids induce eosinophil degranulation by two different mechanisms. Hepatology 33: 582-590. *

• Yokomori H, Oda M, Ogi M, Wakabayashi G, Kawachi S, Yoshimura K, Nagai T, Kitajima M, Nomura M, Hibi T 2005 Expression of adhesion molecules on mature cholangiocytes in canal of Hering and bile ductules in wedge biopsy samples of primary biliary cirrhosis. World J. Gastroenterol. 11: 4382-4389. *

• Yoon JH, Canbay AE, Werneburg NW, Lee SP, Gores GJ 2004 Oxysterols induce cyclooxygenase-2 expression in cholangiocytes: implications for biliary tract carcinogenesis. Hepatology 39: 732-738. *

• Yoon JH, Gores GJ 2003 Diagnosis, staging, and treatment of cholangiocarcinoma. Curr. Treat. Options Gastroenterol. 6: 105-112.

• Yoon YB, Hagey LR, Hofmann AF, Gurantz D, Michelotti EL, Steinbach JH 1986 Effect of side-chain shortening on the physiologic properties of bile acids: hepatic transport and effect on biliary secretion of 23-nor-ursodeoxycholate in rodents. Gastroenterology 90: 837-852.

• Yovchev MI, Grozdanov PN, Joseph B, Gupta S, Dabeva MD 2007 Novel hepatic progenitor cell surface markers in the adult rat liver. Hepatology 45: 139-149. *

• Zamora-Valdes D, Ponciano-Rodriguez G, Chavez-Tapia NC, Mendez-Sanchez N 2005 The endocannabinoid system in chronic liver disease. Ann. Hepatol. 4: 248-254. *

• Zelcer N, Saeki T, Bot I, Kuil A, Borst P 2003 Transport of bile acids in multidrug-resistance-protein 3-overexpressing cells co-transfected with the ileal Na+-dependent bile-acid transporter. Biochem. J. 369: 23-30. *

• Zhai D, Zhao Y, Chen X, Guo J, He H, Yu Q, Yang J, Davey AK, Wang J 2007 Protective effect of glycyrrhizin, glycyrrhetic acid and matrine on acute cholestasis induced by alpha-naphthyl isothiocyanate in rats. Planta Med. 73: 128-133.

• Zsembery A, Jessner W, Sitter G, Spirli C, Strazzabosco M, Graf J 2002 Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells. Hepatology 35: 95-104. *

• Zsembery A, Thalhammer T, Graf J 2000 Bile formation: a concerted action of membrane transporters in hepatocytes and cholangiocytes. News Physiol. Sci. 15: 6-11. *

Number of references = 276

www psc-literature.org