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Items: 19

1.

Primary sclerosing cholangitis is associated with abnormalities in CFTR.

Werlin S, Scotet V, Uguen K, Audrezet MP, Cohen M, Yaakov Y, Safadi R, Ilan Y, Konikoff F, Galun E, Mizrahi M, Slae M, Sayag S, Cohen-Cymberknoh M, Wilschanski M, Ferec C.

J Cyst Fibros. 2018 May 26. pii: S1569-1993(18)30115-2. doi: 10.1016/j.jcf.2018.04.005. [Epub ahead of print]

PMID:
29807875
2.

Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management.

Eaton JE, Talwalkar JA, Lazaridis KN, Gores GJ, Lindor KD.

Gastroenterology. 2013 Sep;145(3):521-36. doi: 10.1053/j.gastro.2013.06.052. Epub 2013 Jul 1. Review.

3.

CFTR dysfunction predisposes to fibrotic liver disease in a murine model.

Martin CR, Zaman MM, Ketwaroo GA, Bhutta AQ, Coronel E, Popov Y, Schuppan D, Freedman SD.

Am J Physiol Gastrointest Liver Physiol. 2012 Aug 15;303(4):G474-81. doi: 10.1152/ajpgi.00055.2012. Epub 2012 Jun 7.

4.

The safety and efficacy of oral docosahexaenoic acid supplementation for the treatment of primary sclerosing cholangitis - a pilot study.

Martin CR, Blanco PG, Keach JC, Petz JL, Zaman MM, Bhaskar KR, Cluette-Brown JE, Gautam S, Sheth S, Afdhal NH, Lindor KD, Freedman SD.

Aliment Pharmacol Ther. 2012 Jan;35(2):255-65. doi: 10.1111/j.1365-2036.2011.04926.x. Epub 2011 Nov 30.

5.

Evolving concepts in primary sclerosing cholangitis.

Krones E, Graziadei I, Trauner M, Fickert P.

Liver Int. 2012 Mar;32(3):352-69. doi: 10.1111/j.1478-3231.2011.02607.x. Epub 2011 Aug 17. Review.

PMID:
22097926
6.

New insights into autoimmune cholangitis through animal models.

Trauner M, Fickert P, Baghdasaryan A, Claudel T, Halilbasic E, Moustafa T, Wagner M, Zollner G.

Dig Dis. 2010;28(1):99-104. doi: 10.1159/000282072. Epub 2010 May 7. Review.

PMID:
20460897
7.

Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2-/- mice.

Halilbasic E, Fiorotto R, Fickert P, Marschall HU, Moustafa T, Spirli C, Fuchsbichler A, Gumhold J, Silbert D, Zatloukal K, Langner C, Maitra U, Denk H, Hofmann AF, Strazzabosco M, Trauner M.

Hepatology. 2009 Jun;49(6):1972-81. doi: 10.1002/hep.22891.

8.

Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis.

Henckaerts L, Jaspers M, Van Steenbergen W, Vliegen L, Fevery J, Nuytten H, Roskams T, Rutgeerts P, Cassiman JJ, Vermeire S, Cuppens H.

J Hepatol. 2009 Jan;50(1):150-7. doi: 10.1016/j.jhep.2008.07.033. Epub 2008 Oct 7.

PMID:
18992954
9.

Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function.

Pall H, Zielenski J, Jonas MM, DaSilva DA, Potvin KM, Yuan XW, Huang Q, Freedman SD.

J Pediatr. 2007 Sep;151(3):255-9. Epub 2007 Jul 24.

PMID:
17719933
10.
11.

Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis.

Gallegos-Orozco JF, E Yurk C, Wang N, Rakela J, Charlton MR, Cutting GR, Balan V.

Am J Gastroenterol. 2005 Apr;100(4):874-8.

PMID:
15784035
12.

The role of cystic fibrosis gene mutations in determining susceptibility to chronic pancreatitis.

Cohn JA, Mitchell RM, Jowell PS.

Gastroenterol Clin North Am. 2004 Dec;33(4):817-37, vii. Review.

PMID:
15528020
13.

Induction of colitis in cftr-/- mice results in bile duct injury.

Blanco PG, Zaman MM, Junaidi O, Sheth S, Yantiss RK, Nasser IA, Freedman SD.

Am J Physiol Gastrointest Liver Physiol. 2004 Aug;287(2):G491-6. Epub 2004 Apr 2.

14.

Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population.

Neri TM, Cavestro GM, Seghini P, Zanelli PF, Zanetti A, Savi M, Podda M, Zuin M, Colombo M, Floreani A, Rosina F, Bianchi Porro G, Strazzabosco M, Okolicsanyi L.

Dig Liver Dis. 2003 Aug;35(8):571-6.

PMID:
14567462
15.

Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis.

Sheth S, Shea JC, Bishop MD, Chopra S, Regan MM, Malmberg E, Walker C, Ricci R, Tsui LC, Durie PR, Zielenski J, Freedman SD.

Hum Genet. 2003 Aug;113(3):286-92. Epub 2003 Jun 3.

PMID:
12783301
16.

Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.

Spirlì C, Fabris L, Duner E, Fiorotto R, Ballardini G, Roskams T, Larusso NF, Sonzogni A, Okolicsanyi L, Strazzabosco M.

Gastroenterology. 2003 Mar;124(3):737-53.

PMID:
12612912
17.

Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis.

Girodon E, Sternberg D, Chazouillères O, Cazeneuve C, Huot D, Calmus Y, Poupon R, Goossens M, Housset C.

J Hepatol. 2002 Aug;37(2):192-7.

PMID:
12127423
18.
19.

Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency.

Waters DL, Dorney SF, Gruca MA, Martin HC, Howman-Giles R, Kan AE, De Silva M, Gaskin KJ.

Hepatology. 1995 Apr;21(4):963-9.

PMID:
7535738

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