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Semin Liver Dis 2010; 30(2): 125-133
DOI: 10.1055/s-0030-1253222
© Thieme Medical Publishers

The Bile Salt Export Pump: Clinical and Experimental Aspects of Genetic and Acquired Cholestatic Liver Disease

Ping Lam1 , Carol J. Soroka1 , James L. Boyer1
  • 1Liver Center, Yale University School of Medicine, New Haven, Connecticut
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Publication History

Publication Date:
26 April 2010 (online)

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ABSTRACT

The primary transporter responsible for bile salt secretion is the bile salt export pump (BSEP, ABCB11), a member of the ATP-binding cassette (ABC) superfamily, which is located at the bile canalicular apical domain of hepatocytes. In humans, BSEP deficiency results in several different genetic forms of cholestasis, which include progressive familial intrahepatic cholestasis type 2 (PFIC2), benign recurrent intrahepatic cholestasis type 2 (BRIC2), as well as other acquired forms of cholestasis such as drug-induced cholestasis (DIC) and intrahepatic cholestasis of pregnancy (ICP). Because bile salts play a pivotal role in a wide range of physiologic and pathophysiologic processes, regulation of BSEP expression has been a subject of intense research. The authors briefly describe the molecular characteristics of BSEP and then summarize what is known about its role in the pathogenesis of genetic and acquired cholestatic disorders, emphasizing experimental observations from animal models and cell culture in vitro systems.

KEYWORDS

Cholestasis - progressive familial intrahepatic cholestasis - bile salt export pump (BSEP) mutations - polymorphisms - ATP-binding cassette (ABC) transporters - trafficking - recycling - ubiquitination

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Ping LamPh.D. 

Liver Center, Yale University School of Medicine

333 Cedar Street/1080 LMP, P.O. Box 208019, New Haven, CT 06510-8019

Email: ping.lam@yale.edu

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