|Other Names||Bile salt export pump, ATP-binding cassette sub-family B member 11, ABCB11, BSEP|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP6110a was selected from the C-term region of human ABCB11 . A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes.|
|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|
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ABCB11 is involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes. It is expressed predominatly, if not exclusively, in the liver, where it is further localized to the canilicular microvilli and to subcanilicular vesicles fo the hepatocytes. Structurally, ABCB11 is a multifunctional polypeptide with two homologus halves, each containing a hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain. Defects in ABCB11 are the cause of progressive familial intrahepatic cholestasis 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.
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).
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