Human Bile Salt Export Pump (ABCB11) ELISA Kit | BSEP elisa kit
Human Bile Salt Export Pump (ABCB11) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Background: ABCB11 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 multidrug resistance. This particular protein is responsible for the transport of taurocholate and other cholate conjugates from hepatocytes (liver cells) to the bile. In humans, the activity of this transporter is the major determinant of bile formation and bile flow.
NCBI and Uniprot Product Information
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: Membrane protein, multi-pass; Membrane protein, integral; Transporter, ABC family; Transporter; Hydrolase
Chromosomal Location of Human Ortholog: 2q24
Cellular Component: intercellular canaliculus; membrane; apical part of cell; integral to plasma membrane; plasma membrane; integral to membrane
Molecular Function: transporter activity; ATPase activity, coupled to transmembrane movement of substances; 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; bile acid metabolic process; transport; canalicular bile acid transport; transmembrane transport
Disease: Cholestasis, Progressive Familial Intrahepatic, 2; Cholestasis, Benign Recurrent Intrahepatic, 2
Research Articles on BSEP
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Product Notes
The Human BSEP abcb11 (Catalog #AAA9714517) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9714517 ELISA Kit recognizes Human BSEP. It is sometimes possible for the material contained within the vial of "Bile Salt Export Pump (ABCB11), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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