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Rabbit Anti-ABCB11 antibody
Rabbit Anti-ABCB11 antibody
ABCBB_HUMAN; Bile salt export pump; BSEP; EC:7.6.2.-; ATP-binding cassette sub-family B member 11; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2;
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  • NO.:SL12440R
    Clonality:Polyclonal
    Immunogen Species:Rabbit
    React Species:(predicted: Human,Mouse,Rat,Rabbit,)
    Applications:IHC-F ICC IF
    concentration:1mg/ml
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Product Name ABCB11
Chinese Name 胆汁酸盐输出泵抗体
Alias ABCBB_HUMAN; Bile salt export pump; BSEP; EC:7.6.2.-; ATP-binding cassette sub-family B member 11; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2;   
Research Area Tumour  Cell biology  Signal transduction  Transporter  The new supersedes the old  
Immunogen Species Rabbit
Clonality Polyclonal
React Species (predicted: Human, Mouse, Rat, Rabbit, )
Applications IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight 146kDa
Cellular localization The cell membrane 
Form Liquid
Concentration 1mg/ml
immunogen KLH conjugated synthetic peptide derived from human ABCB11: 1001-1100/1321 <Cytoplasmic>
Lsotype IgG
Purification affinity purified by Protein A
Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
Product Detail 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]

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:
O95342

Gene ID:
8647

Database links:

Entrez Gene: 8647 Human

Entrez Gene: 27413 Mouse

Entrez Gene: 83569 Rat

SwissProt: O95342 Human

SwissProt: Q9QY30 Mouse

SwissProt: O70127 Rat



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