Soroka Carol J, Boyer James L
Yale University School of Medicine, Department of Internal Medicine, New Haven, CT 06520, United States.
Mol Aspects Med. 2014 Jun;37:3-14. doi: 10.1016/j.mam.2013.05.001. Epub 2013 May 15.
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 intrahepatic 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.
胆盐输出泵（BSEP，ABCB11）是胆汁酸从肝细胞转运至胆道系统的主要转运体。胆汁形成过程中的这一限速步骤对于依赖胆盐的胆汁流动、胆汁酸的肠肝循环以及膳食脂肪的消化至关重要。BSEP的突变与胆汁淤积性疾病相关，如2型进行性家族性肝内胆汁淤积症（PFIC2）、2型良性复发性肝内胆汁淤积症（BRIC2）、药物性胆汁淤积以及妊娠期肝内胆汁淤积症。针对这些病症开发临床治疗方法需要清楚了解BSEP生物合成、转运以及转录和翻译调控的细胞生物学机制。本章将聚焦于这一关键肝细胞膜转运体的分子和细胞生物学方面。
