The bile salt export pump (BSEP) in health and disease.
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.
Medical faculty of the Heinrich-Heine-University of Düsseldorf, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital, Düsseldorf, Germany. kubitz@med.uni-duesseldorf.de
Ralf
R
Kubitz
Carola
C
Dröge
Jan
J
Stindt
Katrin
K
Weissenberger
Dieter
D
Häussinger
D000074020:ATP Binding Cassette Transporter, Subfamily B, Member 11; D018528:ATP-Binding Cassette Transporters / Q000502:physiology*; D002780:Cholestasis, Intrahepatic / Q000209:etiology; D006801:Humans; D008107:Liver Diseases / Q000209:etiology
C093491:ABCB11 protein, human; D000074020:ATP Binding Cassette Transporter, Subfamily B, Member 11; D018528:ATP-Binding Cassette Transporters