产品货号 :  mlR1954

英文名称 :  ABCB11

中文名称 :  胆汁酸盐输出泵/ATP结合盒转运蛋白11抗体

别    名 :  ABC member 16, MDR/TAP subfamily; ABCBB_HUMAN; ATP binding cassette sub family B (MDR/TAP) member 11; ATP binding cassette sub family B member 11; ATP-binding cassette sub-family B member 11; Bile salt export pump; BRIC2; Bsep; PGY4; progressive familial intrahepatic cholestasis 2; Sister of P glycoprotein; Spgp.  

研究领域 :  肿瘤  细胞生物  免疫学  转录调节因子  通道蛋白  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :   Human, Mouse, Rat, Rabbit,

产品应用 :  ELISA=1:500-1000  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  146kDa

细胞定位 :  细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human BSEP:21-120/1321

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed :  PubMed

产品介绍 :   ATP-binding cassette (ABC) transporters are an evolutionarily conserved family of proteins that catalyze the transport of molecules across extra- and intracellular membranes through the energy of ATP hydrolysis. ABC genes comprise seven subfamilies, designated ABC1, Mdr/TAP, MRP, ALD, OABP, GCN20 and White. The secretion of bile salt molecules from blood into bile is a major driving force for bile formation. Bile salt export pump (BSEP) is a member of the Mdr/TAP subfamily of ABC transporters that mediates the transport of bile acids across the hepatocyte canalicular membrane and regulates bile acid-dependent bile secretion. BSEP contains putative phosphorylation sites for protein kinase A, protein kinase C (PKC) and Ca2+-calmodulin dependent kinase II, whose regulation may be dependent on bile salt concentration.

Function:

Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes.

Subunit:

Interacts with HAX1.

Subcellular Location:

Membrane; Multi-pass membrane protein.

Tissue Specificity:

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.

DISEASE:

Defects in ABCB11 are the cause of progressive familial intrahepatic cholestasis type 2 (PFIC2) [MIM:601847]. 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) [MIM:605479]. 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.

Similarity:

Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily. Contains 2 ABC transmembrane type-1 domains. Contains 2 ABC transporter domains.

SWISS:

Q9QY30

Gene ID:

8647

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.