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

Authors
Journal
Clinics and Research in Hepatology and Gastroenterology
2210-7401
Publisher
Elsevier
Volume
36
Issue
6
Identifiers
DOI: 10.1016/j.clinre.2012.06.006
Disciplines
  • Biology
  • Medicine

Abstract

Summary 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.

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