Literature DB >> 22795478

The bile salt export pump (BSEP) in health and disease.

Ralf Kubitz1, Carola Dröge, Jan Stindt, Katrin Weissenberger, Dieter Häussinger.   

Abstract

The bile salt export pump (BSEP) is the major transporter for the secretion of bile acids from hepatocytes into bile in humans. Mutations of BSEP are associated with cholestatic liver diseases of varying severity including progressive familial intrahepatic cholestasis type 2 (PFIC-2), benign recurrent intrahepatic cholestasis type 2 (BRIC-2) and genetic polymorphisms are linked to intrahepatic cholestasis of pregnancy (ICP) and drug-induced liver injury (DILI). Detailed analysis of these diseases has considerably increased our knowledge about physiology and pathophysiology of bile secretion in humans. This review focuses on expression, localization, and function, short- and long-term regulation of BSEP as well as diseases association and treatment options for BSEP-associated diseases.
Copyright © 2012 Elsevier Masson SAS. All rights reserved.

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Year:  2012        PMID: 22795478     DOI: 10.1016/j.clinre.2012.06.006

Source DB:  PubMed          Journal:  Clin Res Hepatol Gastroenterol        ISSN: 2210-7401            Impact factor:   2.947


  51 in total

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Review 9.  Autoimmune BSEP disease: disease recurrence after liver transplantation for progressive familial intrahepatic cholestasis.

Authors:  Ralf Kubitz; Carola Dröge; Stefanie Kluge; Claudia Stross; Nathalie Walter; Verena Keitel; Dieter Häussinger; Jan Stindt
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