ABCB11 blocking peptide

ABCB11 Blocking Peptide (C-term)

• Gene Names: ABCB11; BSEP; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2
• Synonyms: ABCB11; ABCB11 Blocking Peptide (C-term); Bile salt export pump; ATP-binding cassette sub-family B member 11; BSEP; ABCB11 blocking peptide

• Specificity: The synthetic peptide sequence is selected from aa 1083-1097 of HUMAN ABCB11
• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 1321

• Cellular Location: Membrane; Multi-pass membrane protein.
• Tissue Location: Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli and to subcanalicular vesicles of the hepatocytes by in situ
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for ABCB11 blocking peptide

Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes.

NCBI and Uniprot Product Information

• NCBI GI #: 262527527
• NCBI GeneID: 8647
• UniProt Accession #: O95342; Q53TL2; Q9UNB2
• Molecular Weight: 146,407 Da
• NCBI Official Full Name: Bile salt export pump
• NCBI Official Synonym Full Names: ATP binding cassette subfamily B member 11
• NCBI Official Symbol: ABCB11
• NCBI Official Synonym Symbols: BSEP; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2
• NCBI Protein Information: bile salt export pump
• UniProt Protein Name: Bile salt export pump
• Protein Family: ABC transporter B family
• UniProt Gene Name: ABCB11
• UniProt Synonym Gene Names: BSEP
• UniProt Entry Name: ABCBB_HUMAN

NCBI Description

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 Description

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: Hydrolase; Transporter, ABC family; Transporter; Membrane protein, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 2q24

Cellular Component: integral to plasma membrane; membrane; plasma membrane

Molecular Function: bile acid-exporting ATPase activity; canalicular bile acid transmembrane transporter activity; sodium-exporting ATPase activity, phosphorylative mechanism; transporter activity

Biological Process: bile acid and bile salt transport; bile acid biosynthetic process; canalicular bile acid transport; transport

Disease: Cholestasis, Benign Recurrent Intrahepatic, 2; Cholestasis, Progressive Familial Intrahepatic, 2