cystic fibrosis or CFTR, Primary Sclerosing Cholangitis Literature

Primary Sclerosing Cholangitis Literature

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Scientific Literature, cystic fibrosis or CFTR

Abbott J. Health-related quality of life measurement in cystic fibrosis: advances and limitations. Chron. Respir. Dis. 6: 31-41 (2009).
Abbott J, Hart A, Morton AM, Dey P, Conway SP, Webb AK. Can health-related quality of life predict survival in adults with cystic fibrosis? Am. J. Respir. Crit. Care Med. 179: 54-58 (2009). *
Abbott J, Havermans T, Hart A. Adherence to the medical regimen: clinical implications of new findings. Curr. Opin. Pulm. Med. Aug 6 [Epub ahead of print] (2009).
Abbott J, Holt A, Hart A, Morton AM, Macdougall L, Pogson M, Milne G, Rodgers HC, Conway SP. What defines a pulmonary exacerbation? The perceptions of adults with cystic fibrosis. J. Cyst. Fibros. 8: 356-359 (2009).
Abbott J, Morton AM, Musson H, Conway SP, Etherington C, Gee L, Fitzjohn J, Kevin Webb A. Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis. Clin. Nutr. 26: 91-99 (2007).
Abdel Rahman H, Abdul Wahab A, Abdel Rahman MO, Mostafa OA. Faecal elastase-1 concentration in cystic fibrosis patients with CFTR I1234V mutation. Acta Paediatr. 95: 1066-1069 (2006). *
Abdul-Wahab A, Janahi IA, Abdel-Rahman MO. Sweat chloride concentration in cystic fibrosis patients with cystic fibrosis trans-membrane conductance regulator I1234V mutation. Saudi Med. J. 30: 1101-1102 (2009).
Abdulhamid I, Beck FW, Millard S, Chen X, Prasad A. Effect of zinc supplementation on respiratory tract infections in children with cystic fibrosis. Pediatr. Pulmonol. 43: 281-287 (2008).
Abdulhamid I, Wise TL, Andrews S, Biglin K, Lehr VT. Elevated serum tobramycin concentrations after treatment with tobramycin inhalation in a preterm infant. Pharmacotherapy 28: 939-944 (2008).
Abdullaev IF, Rudkouskaya A, Schools GP, Kimelberg HK, Mongin AA. Pharmacological comparison of swelling-activated excitatory amino acid release and Cl- currents in rat cultured astrocytes. J. Physiol. 572: 677-689 (2006). *
Abely M. Treatment of airway inflammation in cystic fibrosis. Arch. Pediatr. 14: 1350-1355 (2007).
Ablin DS, Ziegler M. Ulcerative type of colitis associated with the use of high strength pancreatic enzyme supplements in cystic fibrosis. Pediatr. Radiol. 25: 113-115 (1995).
Abramowsky C, Dahms B, Swinehart G. IgA-associated glomerular deposits in liver disease. Hum. Pathol. 16: 1243-1246 (1985).
Abreu MT. The pathogenesis of inflammatory bowel disease: translational implications for clinicians. Curr. Gastroenterol. Rep. 4: 481-489 (2002).
Abu-Alshiekh NK, Kofahi SM, Nusair ZM. The use of sweat chloride test for screening cystic fibrosis among malnourished children suffering from frequent respiratory infections. Saudi Med. J. 30: 1526-1531 (2009).
Accurso FJ. Update in cystic fibrosis 2006. Am. J. Respir. Crit. Care Med. 175: 754-757 (2007). *
Accurso FJ. Update in cystic fibrosis 2007. Am. J. Respir. Crit. Care Med. 177: 1058-1061 (2008). *
Accurso FJ, Sontag MK. Gene modifiers in cystic fibrosis. J. Clin. Invest. 118: 839-841 (2008). *
Achterbergh R, Lakeman P, Stemerding D, Moors EH, Cornel MC. Implementation of preconceptional carrier screening for cystic fibrosis and haemoglobinopathies: a sociotechnical analysis. Health Policy 83: 277-286 (2007).
Acton JD, Wilmott RW. Phenotype of CF and the effects of possible modifier genes. Paediatr. Respir. Rev. 2: 332-339 (2001).
Adcock IM, Ito K, Barnes PJ. Histone deacetylation: an important mechanism in inflammatory lung diseases. COPD 2: 445-455 (2005).
Adib-Conquy M, Pedron T, Petit-Bertron AF, Tabary O, Corvol H, Jacquot J, Clement A, Cavaillon JM. Neutrophils in cystic fibrosis display a distinct gene expression pattern. Mol. Med. 14: 36-44 (2008). *
Adler AI, Gunn E, Haworth CS, Bilton D. Characteristics of adults with and without cystic fibrosis-related diabetes. Diabet. Med. 24: 1143-1148 (2007). *
Adler AI, Shine BS, Chamnan P, Haworth CS, Bilton D. Genetic determinants and epidemiology of cystic fibrosis-related diabetes - results from a British cohort of children and adults. Diabetes Care 31: 1789-1794 (2008). *
Adler FR, Aurora P, Barker DH, Barr ML, Blackwell LS, Bosma OH, Brown S, Cox DR, Jensen JL, Kurland G, Nossent GD, Quittner AL, Robinson WM, Romero SL, Spencer H, Sweet SC, van der Bij W, Vermeulen J, Verschuuren EA, Vrijlandt EJ, Walsh W, Woo MS, Liou TG. Lung transplantation for cystic fibrosis. Proc. Am. Thorac. Soc. 6: 619-633 (2009). *
Aebi C, Theiler F, Aebischer CC, Schoeni MH. Autoantibodies directed against bactericidal/permeability-increasing protein in patients with cystic fibrosis: association with microbial respiratory tract colonization. Pediatr. Infect. Dis. J. 19: 207-212 (2000).
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 A, Singh KP, Jain A. Medical significance and management of staphylococcal biofilm. FEMS Immunol. Med. Microbiol. Aug 19 [Epub ahead of print] (2009). *
Agarwal R, Aggarwal AN, Gupta D, Bal A, Das A. Case report: a rare cause of miliary nodules - allergic bronchopulmonary aspergillosis. Br. J. Radiol. 82: e151-e154 (2009).
Agarwal R, Aggarwal AN, Gupta N, Gupta D. A rare cause of acute respiratory failure - allergic bronchopulmonary aspergillosis. Mycoses Dec 21 [Epub ahead of print] (2009).
Agarwal R, Singh N, Aggarwal AN. An unusual association between Mycobacterium tuberculosis and Aspergillus fumigatus. Monaldi Arch. Chest Dis. 69: 32-34 (2008).
Agarwal R, Srinivas R, Jindal SK. Allergic bronchopulmonary aspergillosis complicating chronic obstructive pulmonary disease. Mycoses 51: 83-85 (2008).
Agrawal A, Mabalirajan U, Ram A, Ghosh B. Novel approaches for inhibition of mucus hypersecretion in asthma. Recent Pat. Inflamm. Allergy Drug Discov. 1: 188-192 (2007).
Agrons GA, Corse WR, Markowitz RI, Suarez ES, Perry DR. Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics 16: 871-893 (1996). *
Ahmad S, Ahmad A, Dremina ES, Sharov VS, Guo X, Jones TN, Loader JE, Tatreau JR, Perraud AL, Schoeneich C, Randell SH, White CW. Bcl-2 suppresses SERCA2 expression in cystic fibrosis airways: role in oxidant-mediated cell death. Am. J. Respir. Crit. Care Med. 179: 816-826 (2009). *
Ahmed N, Corey M, Forstner G, Zielenski J, Tsui LC, Ellis L, Tullis E, Durie P. Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas. Gut 52: 1159-1164 (2003). *
Ahmed S, Bryant L, Hewison J. 'Balance' is in the eye of the beholder: providing information to support informed choices in antenatal screening via Antenatal Screening Web Resource. Health Expect. 10: 309-320 (2007).
Ahner A, Nakatsukasa K, Zhang H, Frizzell RA, Brodsky JL. Small heat-shock proteins select DeltaF508-CFTR for endoplasmic reticulum-associated degradation. Mol. Biol. Cell 18: 806-814 (2007). *
Ai T, Bompadre SG, Sohma Y, Wang X, Li M, Hwang TC. Direct effects of 9-anthracene compounds on cystic fibrosis transmembrane conductance regulator gating. Pflugers Arch. 449: 88-95 (2004). *
Ai T, Bompadre SG, Wang X, Hu S, Li M, Hwang TC. Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents. Mol. Pharmacol. 65: 1415-1426 (2004). *
Aichele D, Schnare M, Saake M, Rollinghoff M, Gessner A. Expression and antimicrobial function of bactericidal permeability-increasing protein in cystic fibrosis patients. Infect. Immun. 74: 4708-4714 (2006). *
Aimoto T, Uchida E, Nakamura Y, Matsushita A, Katsuno A, Chou K, Kawamoto M, Naito Z, Tajiri T. Multicentric pancreatic intraepithelial neoplasias (PanINs) presenting with the clinical features of chronic pancreatitis. J. Hepatobiliary Pancreat. Surg. 15: 549-553 (2008). *
Ajonuma LC, Chan PK, Ng EH, Fok KL, Wong CH, Tsang LL, Tang XX, Ho LS, Lau MC, Chung CM, He Q, Huang HY, Yang DZ, Rowlands DK, Chung YW, Chan HC. Involvement of cystic fibrosis transmembrane conductance regulator (CFTR) in the pathogenesis of hydrosalpinx induced by Chlamydia trachomatis infection. J. Obstet. Gynaecol. Res. 34: 923-930 (2008).
Ajonuma LC, He Q, Sheung Chan PK, Yu Ng EH, Fok KL, Yan Wong CH, Tsang LL, Ho LS, Lau MC, Huang HY, Yang DZ, Rowlands DK, Tang XX, Zhang XH, Chung YW, Chan HC. Involvement of cystic fibrosis transmembrane conductance regulator in infection-induced edema. Cell Biol. Int. 32: 801-806 (2008). *
Ajonuma LC, Lu Q, Cheung BP, Leung WK, Samaranayake LP, Jin L. Expression and localization of cystic fibrosis transmembrane conductance regulator in human gingiva. Cell Biol. Int. 34: 147-152 (2010).
Ajonuma LC, Lu Q, Cheung BP, Leung WK, Samaranayake LP, Jin L. Expression and localization of cystic fibrosis transmembrane conductance regulator in human gingiva. Cell Biol. Int. Sep 4 [Epub ahead of print] (2009).
Ajonuma LC, Tsang LL, Zhang GH, Wong CH, Lau MC, Ho LS, Rowlands DK, Zhou CX, Ng CP, Chen J, Xu PH, Zhu JX, Chung YW, Chan HC. Estrogen-induced abnormally high CFTR expression results in ovarian hyperstimulation syndrome. Mol. Endocrinol. 19: 3038-3044 (2005). *
Akata D, Akhan O. Liver manifestations of cystic fibrosis. Eur. J. Radiol. 61: 11-17 (2007).
Akiba Y, Furukawa O, Guth PH, Engel E, Nastaskin I, Sassani P, Dukkipatis R, Pushkin A, Kurtz I, Kaunitz JD. Cellular bicarbonate protects rat duodenal mucosa from acid-induced injury. J. Clin. Invest. 108: 1807-1816 (2001). *
Akiba Y, Jung M, Ouk S, Kaunitz JD. A novel small molecule CFTR inhibitor attenuates HCO3- secretion and duodenal ulcer formation in rats. Am. J. Physiol. Gastrointest. Liver Physiol. 289: G753-G759 (2005). *
Akiba Y, Mizumori M, Guth PH, Engel E, Kaunitz JD. Duodenal brush border intestinal alkaline phosphatase activity affects bicarbonate secretion in rats. Am. J. Physiol. Gastrointest. Liver Physiol. 293: G1223-G1233 (2007). *
Al Alam D, Deslee G, Tournois C, Lamkhioued B, Lebargy F, Merten M, Belaaouaj A, Guenounou M, Gangloff SC. Impaired IL-8 chemokine secretion by S. aureus-activated epithelium and T cell chemotaxis in cystic fibrosis. Am. J. Respir. Cell. Mol. Biol. Jul 13 [Epub ahead of print] (2009). *
Al-Abed Y, Hameed W, Roy J, Kumar AP. Appendicitis in an adult patient with cystic fibrosis: a diagnostic challenge. Gut 56: 1799-1800 (2007). *
Al-Nakkash L, Clarke LL, Rottinghaus GE, Chen YJ, Cooper K, Rubin LJ. Dietary genistein stimulates anion secretion across female murine intestine. J. Nutr. 136: 2785-2790 (2006). *
Al-Nakkash L, Springsteel MF, Kurth MJ, Nantz MH. Activation of CFTR by UC(CF)-029 and genistein. Bioorg. Med. Chem. 18: 3874-3877 (2008). *
Al-Shawwa BA, Rao AR. Cystic fibrosis and renal disease: a case report. J. Med. Case Reports 1: 24 (2007). *
Al-Turkmani MR, Andersson C, Alturkmani R, Katrangi W, Cluette-Brown JE, Freedman SD, Laposata M. A mechanism accounting for the low cellular level of linoleic acid in cystic fibrosis and its reversal by DHA. J. Lipid Res. 49: 1946-1954 (2008). *
Al-Turkmani MR, Freedman SD, Laposata M. Fatty acid alterations and n-3 fatty acid supplementation in cystic fibrosis. Prostaglandins Leukot. Essent. Fatty Acids 77: 309-318 (2007). *
Alaminos M, Gonzalez-Andrades M, Munoz-Avila JI, Garzon I, Sanchez-Quevedo MC, Campos A. Volumetric and ionic regulation during the in vitro development of a corneal endothelial barrier. Exp. Eye Res. 86: 758-769 (2008).
Alazmi WM, Fogel EL, Schmidt S, Watkins JL, McHenry L, Sherman S, Lehman GA. ERCP findings in idiopathic pancreatitis: patients who are cystic fibrosis gene positive and negative. Gastrointest. Endosc. 63: 234-239 (2006).
Alberti S, Bohse K, Arndt V, Schmitz A, Hohfeld J. The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator. Mol. Biol. Cell 15: 4003-4010 (2004). *
Albini S, Abril C, Franchini M, Hussy D, Filioussis G. Stenotrophomonas maltophilia isolated from the airways of animals with chronic respiratory disease. Schweiz. Arch. Tierheilkd. 151: 323-328 (2009).
Albrecht LA, Gorges SW, Styne DM, Bremer AA. Shwachman-Diamond syndrome presenting as hypoglycemia. Clin. Pediatr. (Phila.) 48: 212-214 (2009).
Aldamiz-Echevarria L, Prieto JA, Andrade F, Elorz J, Sojo A, Lage S, Sanjurjo P, Vazquez C, Rodriguez-Soriano J. Persistence of essential fatty acid deficiency in cystic fibrosis despite nutritional therapy. Pediatr. Res. 66: 585-589 (2009).
Aleksandrov AA, Aleksandrov LA, Riordan JR. CFTR (ABCC7) is a hydrolyzable-ligand-gated channel. Pflugers Arch. 453: 693-702 (2007). *
Aleksandrov AA, Cui L, Riordan JR. Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator. J. Physiol. 587: 2875-2886 (2009). *
Aleksandrov LA, Aleksandrov AA, Riordan JR. Mg2+ dependent ATP occlusion at the first nucleotide binding domain (NBD1) of CFTR does not require the second (NBD2). Biochem. J. 416: 129-136 (2008). *
Alexander BD, Petzold EW, Reller LB, Palmer SM, Davis RD, Woods CW, Lipuma JJ. Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia cepacia complex. Am. J. Transplant. 8: 1025-1030 (2008).
Alexander C, Ivetac AD, Liu X, Norimatsu Y, Serrano JR, Landstrom A, Sansom MS, Dawson DC. Cystic fibrosis transmembrane conductance regulator: using differential reactivity toward channel-permeant and channel-impermeant thiol-reactive probes to test a molecular model for the pore. Biochemistry 48: 10078-10088 (2009). *
Alexander CL, Urbanski SJ, Hilsden R, Rabin H, Macnaughton WK, Beck PL. The risk of gastrointestinal malignancies in cystic fibrosis. Case report of a patient with a near obstructing villous adenoma found on colon cancer screening and Barrett's esophagus. J. Cyst. Fibros. 7: 1-6 (2008).
Alfaro M, Tapadinhas F, Neves A, Costa Trindade J. Atopy and autoimmunity - a case report. Rev. Port. Pneumol. 13: 729-735 (2007).
Alfonso-Sanchez MA, Perez-Miranda AM, Garcia-Obregon S, Pena JA. An evolutionary approach to the high frequency of the Delta F508 CFTR mutation in European populations. Med. Hypotheses Jan 26 [Epub ahead of print] (2010).
Algar FJ, Cano JR, Moreno P, Espinosa D, Cerezo F, Alvarez A, Baamonde C, Santos F, Vaquero JM, Salvatierra A. Results of lung transplantation in patients with cystic fibrosis. Transplant. Proc. 40: 3085-3087 (2008). *
Algar FJ, Moreno P, Cano JR, Espinosa D, Alvarez A, Cerezo F, Baamonde C, Vaquero JM, Santos F, Salvatierra A. Urgency-code lung transplantation for cystic fibrosis: experience and results. Transplant. Proc. 40: 3067-3069 (2008). *
Alghisi F, Angioni A, Tomaiuolo AC, D'Apice MR, Bella S, Novelli G, Lucidi V. Diagnosis of atypical CF: a case-report to reflect. J. Cyst. Fibros. 7: 292-294 (2008).
Alghisi F, Bella S, Lucidi V, Angioni A, Tomaiuolo AC, D'Apice MR, Gambardella S, Novelli G. Phenotypic variability in a family with pancreatitis and cystic fibrosis sharing common mild CFTR mutation: report on CFTR mutations and their phenotypic variability. Pancreas 38: 109-110 (2009).
Alhanout K, Brunel JM, Raoult D, Rolain JM. In vitro antibacterial activity of aminosterols against multidrug-resistant bacteria from patients with cystic fibrosis. J. Antimicrob. Chemother. 64: 810-814 (2009).
Alhede M, Bjarnsholt T, Jensen PO, Phipps RK, Moser C, Christophersen L, Christensen LD, van Gennip M, Parsek M, Hoiby N, Rasmussen TB, Givskov M. Pseudomonas aeruginosa recognizes and responds aggressively to the presence of polymorphonuclear leukocytes. Microbiology 155: 3500-3508 (2009).
Ali A, Zayed H, Wilkins J, Wyatt H, Rashid H. Endovascular port-a-cath insertion in a cystic fibrosis patient with occluded superior vena cava. Vasc. Endovascular Surg. 43: 280-283 (2009).
Ali BR. Is cystic fibrosis-related diabetes an apoptotic consequence of ER stress in pancreatic cells? Med. Hypotheses 72: 55-57 (2009).
Ali MS, Pearson JP. Upper airway mucin gene expression: a review. Laryngoscope 117: 932-938 (2007).
Alibakhshi R, Kianishirazi R, Cassiman JJ, Zamani M, Cuppens H. Analysis of the CFTR gene in Iranian cystic fibrosis patients: identification of eight novel mutations. J. Cyst. Fibros. 7: 102-109 (2008).
Alibakhshi R, Zamani M. Mutation analysis of CFTR gene in 70 Iranian cystic fibrosis patients. Iran. J. Allergy Asthma Immunol. 5: 3-8 (2006).
Alipour M, Suntres ZE, Halwani M, Azghani AO, Omri A. Activity and interactions of liposomal antibiotics in presence of polyanions and sputum of patients with cystic fibrosis. PLoS ONE 4: e5724 (2009). *
Alipour M, Suntres ZE, Omri A. Importance of DNase and alginate lyase for enhancing free and liposome encapsulated aminoglycoside activity against Pseudomonas aeruginosa. J. Antimicrob. Chemother. 64: 317-325 (2009).
Allard JB, Poynter ME, Marr KA, Cohn L, Rincon M, Whittaker LA. Aspergillus fumigatus generates an enhanced Th2-biased immune response in mice with defective cystic fibrosis transmembrane conductance regulator. J. Immunol. 177: 5186-5194 (2006). *
Allen J, Visner G. Lung transplantation in cystic fibrosis - primum non nocere? N. Engl. J. Med. 357: 2186-2188 (2007). *
Almstrand AC, Ljungstrom E, Lausmaa J, Bake B, Sjovall P, Olin AC. Airway monitoring by collection and mass spectrometric analysis of exhaled particles. Anal. Chem. 81: 662-668 (2009). *
Alonso MJ, Heine-Suner D, Calvo M, Rosell J, Gimenez J, Ramos MD, Telleria JJ, Palacio A, Estivill X, Casals T. Spectrum of mutations in the CFTR gene in cystic fibrosis patients of Spanish ancestry. Ann. Hum. Genet. 71: 194-201 (2007). *
Alper OM, Wong LJ, Young S, Pearl M, Graham S, Sherwin J, Nussbaum E, Nielson D, Platzker A, Davies Z, Lieberthal A, Chin T, Shay G, Hardy K, Kharrazi M. Identification of novel and rare mutations in California Hispanic and African American cystic fibrosis patients. Hum. Mutat. 24: 353 (2004). *
Alper SL, Stewart AK, Chernova MN, Zolotarev AS, Clark JS, Vandorpe DH. Anion exchangers in flux: functional differences between human and mouse SLC26A6 polypeptides. Novartis Found. Symp. 273: 107-119 (2006).
Altes TA, Eichinger M, Puderbach M. Magnetic resonance imaging of the lung in cystic fibrosis. Proc. Am. Thorac. Soc. 4: 321-327 (2007). *
Altman E, Wang Z, Aaron SD, Liu X, Vandemheen KL, Ferris W, Giesbrecht T, Li J. Epidemiological investigation and glycotyping of clinical Pseudomonas aeruginosa isolates from patients with cystic fibrosis by mass spectrometry: association with multiple drug resistance. J. Microbiol. Methods 76: 204-208 (2009). *
Alton E. Progress and prospects: gene therapy clinical trials (part 1). Gene Ther. 14: 1439-1447 (2007). *
Alton EW, Davies JC, Geddes DM. Biomarkers for cystic fibrosis: are we progressing? Am. J. Respir. Crit. Care Med. 175: 750-751 (2007). *
Altuntas B, Gul H, Yarali N, Ertan U. Etiology of chronic diarrhea. Indian J. Pediatr. 66: 657-661 (1999).
Alvarez F. Treatments in chronic cholestasis in children. Ann. Nestle [Engl.] 66: 127-135 (2008).
Alvarez-Ortega C, Harwood CS. Responses of Pseudomonas aeruginosa to low oxygen indicate that growth in the cystic fibrosis lung is by aerobic respiration. Mol. Microbiol. 65: 153-165 (2007). *
Alves C, Lima DS, Cardeal M, Santana A. Low prevalence of glucose intolerance in racially mixed children with cystic fibrosis. Pediatr. Diabetes Feb 8 [Epub ahead of print] (2010).
Alves Cde A, Aguiar RA, Alves AC, Santana MA. Diabetes mellitus in patients with cystic fibrosis. J. Bras. Pneumol. 33: 213-221 (2007). *
Alves Cde A, Lima DS. Cystic fibrosis-related dyslipidemia. J. Bras. Pneumol. 34: 829-837 (2008).
Alwitry A. Vitamin A deficiency in coeliac disease. Br. J. Ophthalmol. 84: 1079-1080 (2000). *
Amadori A, Antonelli A, Balteri I, Schreiber A, Bugiani M, De Rose V. Recurrent exacerbations affect FEV(1) decline in adult patients with cystic fibrosis. Respir. Med. 103: 407-413 (2009).
Amaral MD. Processing of CFTR: traversing the cellular maze - how much CFTR needs to go through to avoid cystic fibrosis? Pediatr. Pulmonol. 39: 479-491 (2005). *
Amaral MD. CFTR and chaperones: processing and degradation. J. Mol. Neurosci. 23: 41-48 (2004).
Amaral MD, Clarke LA, Ramalho AS, Beck S, Broackes-Carter F, Rowntree R, Mouchel N, Williams SH, Harris A, Tzetis M, Steiner B, Sanz J, Gallati S, Nissim-Rafinifa M, Kerem B, Hefferon T, Cutting GR, Goina E, Pagani F. Quantitative methods for the analysis of CFTR transcripts/splicing variants. J. Cyst. Fibros. 3 Suppl. 2: 17-23 (2004).
Amaral MD, Kunzelmann K. Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis. Trends Pharmacol. Sci. 28: 334-341 (2007). *
Ameen N, Alexis J, Salas P. Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract. Histochem. Cell Biol. 114: 69-75 (2000). *
Ameen N, Apodaca G. Defective CFTR apical endocytosis and enterocyte brush border in myosin VI-deficient mice. Traffic 8: 998-1006 (2007). *
Ameen N, Silvis M, Bradbury NA. Endocytic trafficking of CFTR in health and disease. J. Cyst. Fibros. 6: 1-14 (2007). *
Ameen NA, Figueroa Y, Salas PJ. Anomalous apical plasma membrane phenotype in CK8-deficient mice indicates a novel role for intermediate filaments in the polarization of simple epithelia. J. Cell Sci. 114: 563-575 (2001). *
Ameen NA, Salas PJ. Microvillus inclusion disease: a genetic defect affecting apical membrane protein traffic in intestinal epithelium. Traffic 1: 76-83 (2000). *
Amin R, Dupuis A, Aaron SD, Ratjen F. The effect of chronic infection with Aspergillus fumigatus on lung function and hospitalization in cystic fibrosis patients. Chest Jun 30 [Epub ahead of print] (2009).
Amin R, Noone PG, Ratjen F. Chemical pleurodesis versus surgical intervention for persistent and recurrent pneumothoraces in cystic fibrosis. Cochrane Database Syst. Rev. 2: CD007481 (2009). *
Amin R, Ratjen F. Cystic fibrosis: a review of pulmonary and nutritional therapies. Adv. Pediatr. 55: 99-121 (2008).
Amirav I, Cohen-Cymberknoh M, Shoseyov D, Kerem E. Primary ciliary dyskinesia: prospects for new therapies, building on the experience in cystic fibrosis. Paediatr. Respir. Rev. 10: 58-62 (2009).
Amos J, Grody W. Development and integration of molecular genetic tests into clinical practice: the US experience. Expert Rev. Mol. Diagn. 4: 465-477 (2004).
Amos JA, Bridge-Cook P, Ponek V, Jarvis MR. A universal array-based multiplexed test for cystic fibrosis carrier screening. Expert Rev. Mol. Diagn. 6: 15-22 (2006).
Ananthakrishnan AN, Saeian K. Caroli's disease: identification and treatment strategy. Curr. Gastroenterol. Rep. 9: 151-155 (2007).
Anbar RD. Lung transplantation and survival in children with cystic fibrosis. N. Engl. J. Med. 358: 1754 (2008).
Andersen DH. The present diagnosis and therapy of cystic fibrosis of the pancreas. Proc. R. Soc. Med. 42: 25-32 (1949). *
Andersen HU, Lanng S, Pressler T, Laugesen CS, Mathiesen ER. Cystic fibrosis-related diabetes: the presence of microvascular diabetes complications. Diabetes Care 29: 2660-2663 (2006). *
Anderson CA, Palmer CA, Ney AL, Becker B, Schaffel SD, Quickel RR. Evaluation of the safety of high-frequency chest wall oscillation (HFCWO) therapy in blunt thoracic trauma patients. J. Trauma Manag. Outcomes 2: 8 (2008). *
Anderson CM, Howard A, Walters JR, Ganapathy V, Thwaites DT. Taurine uptake across the human intestinal brush-border membrane is via two transporters: H+-coupled PAT1 (SLC36A1) and Na+ and Cl- dependent TauT (SLC6A6). J. Physiol. 587: 731-744 (2009). *
Anderson GG, Moreau-Marquis S, Stanton BA, O'Toole GA. In vitro analysis of tobramycin-treated Pseudomonas aeruginosa biofilms on cystic fibrosis-derived airway epithelial cells. Infect. Immun. 76: 1423-1433 (2008). *
Anderson GG, Yahr TL, Lovewell RR, O'Toole GA. The Pseudomonas aeruginosa magnesium transporter MgtE inhibits transcription of the type III secretion system. Infect. Immun. Dec 22 [Epub ahead of print] (2009). *
Anderson RD, Roddam LF, Bettiol S, Sanderson K, Reid DW. Biosignificance of bacterial cyanogenesis in the CF lung. J. Cyst. Fibros. Feb 13 [Epub ahead of print] (2010).
Anderson SW, Stapp JR, Burns JL, Qin X. Characterization of small colony variant Stenotrophomonas maltophilia isolated from the sputum specimens of five patients with cystic fibrosis. J. Clin. Microbiol. 45: 529-535 (2007). *
Andersson C, Al-Turkmani MR, Savaille JE, Alturkmani R, Katrangi W, Cluette-Brown JE, Zaman MM, Laposata M, Freedman SD. Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism. J. Lipid Res. 49: 1692-1700 (2008). *
Andersson C, Dragomir A, Hjelte L, Roomans GM. Cystic fibrosis transmembrane conductance regulator (CFTR) activity in nasal epithelial cells from cystic fibrosis patients with severe genotypes. Clin. Sci. (Lond.) 103: 417-424 (2002). *
Andersson C, Gaston B, Roomans GM. S-Nitrosoglutathione induces functional DeltaF508-CFTR in airway epithelial cells. Biochem. Biophys. Res. Commun. 297: 552-557 (2002). *
Andersson C, Zaman MM, Jones AB, Freedman SD. Alterations in immune response and PPAR/LXR regulation in cystic fibrosis macrophages. J. Cyst. Fibros. 7: 68-78 (2008).
Andresen D. Microbiological diagnostic procedures in respiratory infections: mycobacterial infections. Paediatr. Respir. Rev. 8: 221-230 (2007).
Angulo P. Use of ursodeoxycholic acid in patients with liver disease. Curr. Gastroenterol. Rep. 4: 37-44 (2002).
Anil N, Singh M, Rajwanshi A, Vohra H. Induced sputum nitrites correlate with FEV in children with cystic fibrosis. Acta Paediatr. Jan 21 [Epub ahead of print] (2010).
Annese V, Latiano A, Rossi L, Lombardi G, Dallapiccola B, Serafini S, Damonte G, Andriulli A, Magnani M. Erythrocytes-mediated delivery of dexamethasone in steroid-dependent IBD patients - a pilot uncontrolled study. Am. J. Gastroenterol. 100: 1370-1375 (2005). *
Anson DS, Smith GJ, Parsons DW. Gene therapy for cystic fibrosis airway disease- is clinical success imminent? Curr. Gene Ther. 6: 161-179 (2006).
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).
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