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

产品简要

公司名称 :

MyBioSource

产品类型 :

抗体

产品名称 :

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

MBS603822

规格 :

0.1毫克

价格 :

520美元

克隆性 :

单克隆

宿主 :

小鼠

共轭标签 :

未共轭

克隆名称 :

6D172

反应物种 :

人类

应用 :

免疫印迹, 免疫细胞化学

更多信息或购买 :

MyBioSource 产品网页

产品信息

目录号 :

MBS603822

产品类型 :

抗体

产品全称 :

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

产品简称 :

BSEP

产品名称同义词 :

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

其他名称 :

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

其他基因名称 :

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

UniProt数据库进入名 :

ABCBB_HUMAN

克隆性 :

单克隆

抗体亚型 :

IgG1

克隆 :

6D172

宿主 :

小鼠

反应物种 :

人类

特异性 :

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

纯度 :

Affinity Purified. Purified by Protein G affinity chromatography.

形式 :

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

储存稳定性 :

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.

检测过的应用 :

免疫印迹(免疫印迹), 免疫细胞化学(免疫细胞化学)

应用笔记 :

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

其它信息1 :

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

产品种类 :

Antibodies; Abs to Proteins

产品描述 :

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.

NCBI GI登录号 :

21536378

NCBI登录号 :

NP_003733.2

NCBI基因登录号 :

NM_003742.2

UniProt数据库登录号 :

O95342

NCBI分子量 :

146,407 Da

NCBI信号通路 :

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)

NCBI总结 :

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]

UniProt数据库总结 :

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

尺寸1 :

0.1毫克

价格1 :

520美元

更多信息或购买 :

MyBioSource 产品网页

公司信息