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

被引:151
作者
Kubitz, Ralf [1 ]
Droege, Carola [1 ]
Stindt, Jan [1 ]
Weissenberger, Katrin [1 ]
Haeussinger, Dieter [1 ]
机构
[1] Univ Dusseldorf, Fac Med, Dept Gastroenterol Hepatol & Infect Dis, Univ Hosp, D-40225 Dusseldorf, Germany
关键词
FAMILIAL INTRAHEPATIC CHOLESTASIS; PROTEIN-KINASE-C; FARNESOID-X-RECEPTOR; MULTIDRUG-RESISTANCE PROTEIN-2; INDUCED LIVER-INJURY; ESTRADIOL 17-BETA-D-GLUCURONIDE-INDUCED CHOLESTASIS; TAUROURSODEOXYCHOLIC ACID INSERTS; EXTRACORPOREAL ALBUMIN DIALYSIS; EXTERNAL BILIARY DIVERSION; ORGANIC ANION TRANSPORTERS;
D O I
10.1016/j.clinre.2012.06.006
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
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. (c) 2012 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码:536 / 553
页数:18
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