| Applications: | WB, IHC-P |
| Species Reactivity: | Human, Mouse |
| Isotype: | Rabbit Ig |
| Purification: | Purified Rabbit Polyclonal Antibody |
| Description: | 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. |
| Further Information: | This ABCB11 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1038-1067 amino acids from the C-terminal region of human ABCB11. |
| Synonym: | Bile salt export pump, ATP-binding cassette sub-family B member 11, ABCB11, BSEP |
| Ascension Number: | O95342 |
| NCBI EntrezGene: | 8647 |
| Shipping Conditions: | Ship on cold packs |
| Storage: | Maintain refrigerated at 2-8 C for up to 2 weeks. For long term storage store at -20 C in small aliquots to prevent freeze-thaw cycles. |
| Usage: | Research use only |
