NP_003733.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
bile salt export pump
NCBI Official Synonym Full Names
ATP-binding cassette, sub-family B (MDR/TAP), member 11
NCBI Official Synonym Symbols
BSEP; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2 [Similar Products]
NCBI Protein Information
bile salt export pump
UniProt Protein Name
Bile salt export pump
UniProt Synonym Protein Names
ATP-binding cassette sub-family B member 11
UniProt Synonym Gene Names
UniProt Entry Name
ABCBB_HUMAN
NCBI Summary for ABCB11
The membrane-associated protein encoded by this gene 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. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. [provided by RefSeq, Jul 2008]
UniProt Comments for ABCB11
ABCB11: Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes. Defects in ABCB11 are the cause of progressive familial intrahepatic cholestasis type 2 (PFIC2). PFIC2 is an inherited liver disease of childhood which is characterized by cholestasis and normal serum gamma-glutamyltransferase activity. Defects in ABCB11 are also found in cases of chronic intrahepatic cholestasis without obvious familial history of chronic liver disease. Defects in ABCB11 are the cause of benign recurrent intrahepatic cholestasis type 2 (BRIC2). BRIC is characterized by intermittent episodes of cholestasis without progression to liver failure. There is initial elevation of serum bile acids, followed by cholestatic jaundice which generally spontaneously resolves after periods of weeks to months. The cholestatic attacks vary in severity and duration and patients are asymptomatic between episodes, both clinically and biochemically. Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily.
Protein type: Transporter; Membrane protein, multi-pass; Membrane protein, integral; Hydrolase; Transporter, ABC family
Chromosomal Location of Human Ortholog: 2q24
Cellular Component: intercellular canaliculus; membrane; integral to plasma membrane; apical part of cell; plasma membrane; integral to membrane
Molecular Function: ATPase activity, coupled to transmembrane movement of substances; transporter activity; canalicular bile acid transmembrane transporter activity; bile acid-exporting ATPase activity; sodium-exporting ATPase activity, phosphorylative mechanism; ATP binding
Biological Process: bile acid and bile salt transport; bile acid biosynthetic process; transport; bile acid metabolic process; canalicular bile acid transport; transmembrane transport
Disease: Cholestasis, Progressive Familial Intrahepatic, 2; Cholestasis, Benign Recurrent Intrahepatic, 2
Product References and Citations for anti-ABCB11 antibody
Chen, H.L., et al., J. Pediatr. 140(1):119-124 (2002). Saito, S., et al., J. Hum. Genet. 47(1):38-50 (2002). Strautnieks, S.S., et al., Nat. Genet. 20(3):233-238 (1998).
Research Articles on ABCB11
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Pathways associated with anti-ABCB11 antibody
Diseases associated with anti-ABCB11 antibody
Organs/Tissues associated with anti-ABCB11 antibody
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