ABCB11

ATP-binding cassette, sub-family B member 11 also known as ABCB11 is a is_associated_with::protein which in humans is encoded by the ABCB11 is_associated_with::gene.

Function
The product of the ABCB11 gene is an ABC transporter named BSEP (Bile Salt Export Pump), or sPgp (sister of P-glycoprotein). This membrane-associated protein is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White).

This protein is a member of the MDR/TAP subfamily. Some members of the MDR/TAP subfamily are involved in is_associated_with::multidrug resistance. This particular protein is responsible for the transport of taurocholate and other cholate conjugates from is_associated_with::hepatocytes (liver cells) to the is_associated_with::bile. In humans, the activity of this transporter is the major determinant of bile formation and bile flow.

Clinical significance
ABCB11 is a gene associated with is_associated_with::progressive familial intrahepatic cholestasis type 2 (PFIC2). PFIC2 caused by mutations in the ABCB11 gene increases the risk of is_associated_with::hepatocellular carcinoma in early life.