期刊
MOLECULAR ASPECTS OF MEDICINE
卷 37, 期 -, 页码 3-14出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.mam.2013.05.001
关键词
Bile salt secretion; ATP-binding cassette transporter; Cholestasis
资金
- USPHS [R37 DK25636, P30 DK 34989]
The bile salt export pump (BSEP, ABCB11) is the primary transporter of bile acids from the hepatocyte to the biliary system. This rate-limiting step in bile formation is essential to the formation of bile salt dependent bile flow, the enterohepatic circulation of bile acids, and the digestion of dietary fats. Mutations in BSEP are associated with cholestatic diseases such as progressive familial intrahepatic cholestasis type 2 (PFIC2), benign recurrent intrahepatic cholestasis type 2 (BRIC2), drug-induced cholestasis, and intrahephtic cholestasis of pregnancy. Development of clinical therapies for these conditions necessitates a clear understanding of the cell biology of biosynthesis, trafficking, and transcriptional and translational regulation of BSEP. This chapter will focus on the molecular and cell biological aspects of this critical hepatic membrane transporter. (C) 2013 Elsevier Ltd. All rights reserved.
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