Family

Bile salt export pump (IPR030278)

Short name: BSEP

Family relationships

None.

Description

Bile salt export pump (BSEP) is also known as ATP-binding cassette sub-family B member 11 (ABCB11). It is a major transporter responsible for biliary bile acid secretion [PMID: 25085279]. Disruption of the receptor is a risk factor for intrahepatic cholestasis of pregnancy (ICP), a liver disorder associated with increased risk of intrauterine foetal death and prematurity [PMID: 15077010]. Mutations in the ABCB11 gene also cause progressive familial intrahepatic cholestasis type 2 (PFIC2), which is characterised by cholestasis, accompanied with either normal or increased serum gamma-glutamyltransferase activity [PMID: 10579978, PMID: 25085279].

GO terms

Biological Process

GO:0015721 bile acid and bile salt transport

Molecular Function

GO:0005524 ATP binding
GO:0042626 ATPase activity, coupled to transmembrane movement of substances

Cellular Component

GO:0016021 integral component of membrane

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
PANTHER