Signal Transduction Antibodies 1
Anti-ABCB11 Antibody (CAB14694)
- SKU:
- CAB14694
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Description
Antibody Name: | Anti-ABCB11 Antibody |
Antibody SKU: | CAB14694 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IF |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 1050-1150 of human ABCB11 (NP_003733.2). |
Application: | WB IF |
Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | Rat liver |
Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 1050-1150 of human ABCB11 (NP_003733.2). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | RFFQ LLDR QPPI SVYN TAGE KWDN FQGK IDFV DCKF TYPS RPDS QVLN GLSV SISP GQTL AFVG SSGC GKST SIQL LERF YDPD QGKV MIDG HDSK KVNV Q |
Gene ID: | 8647 |
Uniprot: | O95342 |
Cellular Location: | Membrane, Multi-pass membrane protein |
Calculated MW: | 146kDa |
Observed MW: | 146kDa |
Synonyms: | ABCB11, ABC16, BRIC2, BSEP, PFIC-2, PFIC2, PGY4, SPGP |
Background: | 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. |
UniProt Protein Function: | 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. |
UniProt Protein Details: | 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 |
NCBI Summary: | 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 Code: | O95342 |
NCBI GenInfo Identifier: | 262527527 |
NCBI Gene ID: | 8647 |
NCBI Accession: | O95342.2 |
UniProt Related Accession: | O95342 |
Molecular Weight: | |
NCBI Full Name: | Bile salt export pump |
NCBI Synonym Full Names: | ATP binding cassette subfamily B member 11 |
NCBI Official Symbol: | ABCB11 |
NCBI Official Synonym Symbols: | BSEP; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2 |
NCBI Protein Information: | bile salt export pump |
UniProt Protein Name: | Bile salt export pump |
UniProt Synonym Protein Names: | ATP-binding cassette sub-family B member 11 |
UniProt Gene Name: | ABCB11 |
UniProt Entry Name: | ABCBB_HUMAN |