antibody

BSEP (Bile Salt Export Pump, SPGP, Sister of P-glycoprotein, ABCB11)

MBS603822

0.1 mg

520 USD

monoclonal

mouse

nonconjugated

6D172

western blot, immunocytochemistry

Antibody

BSEP (Bile Salt Export Pump, SPGP, Sister of P-glycoprotein, ABCB11)

BSEP

Anti -BSEP (Bile Salt Export Pump, SPGP, Sister of P-glycoprotein, ABCB11)

bile salt export pump; Bile salt export pump; bile salt export pump; OTTHUMP00000204927; sister p-glycoprotein; ABC member 16, MDR/TAP subfamily; ATP-binding cassette sub-family B member 11; progressive familial intrahepatic cholestasis 2; ATP-binding cassette, sub-family B (MDR/TAP), member 11; ATP-binding cassette sub-family B member 11

ABCB11; ABCB11; BSEP; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2; BSEP

ABCBB_HUMAN

Monoclonal

IgG1

6D172

Mouse

Human

Recognizes human BSEP. Does not cross-react with human P-glycoprotein.

Affinity Purified. Purified by Protein G affinity chromatography.

Supplied as a liquid, 0.7% BSA, 100nM sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB), Immunocytochemistry (ICC)

Suitable for use in Immunocytochemistry and Western Blot. Dilution: Western Blot: 1:50-1:200. Immunocytochemistry: Cytospin preparations

Immunogen: Synthetic peptide corresponding to aa705-718, Y705LVHEPPLAVVDHK718 of human BSEP (bile salt export pump).

Antibodies; Abs to Proteins

BSEP 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, BSEP is a multifunctional polypeptide with two homologus halves, each containing a hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain. Defects in BSEP 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 BSEP are also found in cases of chronic intrahepatic cholestasis without obvious familial history of chronic liver disease.

21536378

NP_003733.2

NM_003742.2

O95342

146,407 Da

ABC Transporters Pathway (83035); ABC Transporters Pathway (436); Bile Acid And Bile Salt Metabolism Pathway (106144); Bile Secretion Pathway (193146); Bile Secretion Pathway (193095); Metabolism Of Lipids And Lipoproteins Pathway (160976); Nuclear Receptors In Lipid Metabolism And Toxicity Pathway (198887); Recycling Of Bile Acids And Salts Pathway (106149); Synthesis Of Bile Acids And Bile Salts Pathway (106145); Synthesis Of Bile Acids And Bile Salts Via 7alpha-hydroxycholesterol Pathway (106146)

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]

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: intercellular canaliculus; membrane; apical part of cell; integral to plasma membrane; integral to membrane; plasma 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; transport; bile acid metabolic process; canalicular bile acid transport; transmembrane transport. Disease: Cholestasis, Progressive Familial Intrahepatic, 2; Cholestasis, Benign Recurrent Intrahepatic, 2

0.1 mg

520 USD