ABCB11 polyclonal antibody
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Specification
Product Description
Rabbit polyclonal antibody raised against partial recombinant human ABCB11.
Immunogen
Recombinant protein corresponding to amino acids 616-746 of human ABCB11.
Sequence
RLSTVRAADTIIGFEHGTAVERGTHEELLERKGVYFTLVTLQSQGNQALNEEDIKDATEDDMLARTFSRGSYQDSLRASIRQRSKSQLSYLVHEPPLAVVDHKSTYEEDRKDKDIPVQEEVEPAPVRRILK
Host
Rabbit
Reactivity
Human
Form
Liquid
Purification
Antigen affinity purification
Isotype
IgG
Recommend Usage
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (1:2500-1:5000)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS, pH 7.2 (40% glycerol, 0.02% sodium azide).
Storage Instruction
Store at 4°C for short term storage. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining (Formalin-fixed paraffin-embedded sections) of human liver shows moderate membranous positivity in hepatocytes. -
Gene Info — ABCB11
Entrez GeneID
8647Protein Accession#
O95342Gene Name
ABCB11
Gene Alias
ABC16, BSEP, PFIC-2, PFIC2, PGY4, SPGP
Gene Description
ATP-binding cassette, sub-family B (MDR/TAP), member 11
Gene Ontology
HyperlinkGene 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
Other Designations
ABC member 16, MDR/TAP subfamily|bile salt export pump|progressive familial intrahepatic cholestasis 2|sister p-glycoprotein
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Interactome
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Pathway
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Disease
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Publication Reference
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Mutations in TJP2 cause progressive cholestatic liver disease.
Sambrotta M, Strautnieks S, Papouli E, Rushton P, Clark BE, Parry DA, Logan CV, Newbury LJ, Kamath BM, Ling S, Grammatikopoulos T, Wagner BE, Magee JC, Sokol RJ, Mieli-Vergani G; University of Washington Center for Mendelian Genomics, Smith JD, Johnson CA, McClean P, Simpson MA, Knisely AS, Bull LN, Thompson RJ.
Nature Genetics 2014 Apr; 46(4):326.
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Polymorphisms in ABCB11 and ATP8B1 Associated with Development of Severe Intrahepatic Cholestasis in Hodgkin's Lymphoma.
Blackmore L, Knisely AS, Hartley JL, McKay K, Gissen P, Marcus R, Shawcross DL.
Journal of Clinical and Experimental Hepatology 2013 Jun; 3(2):159.
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Mutations in TJP2 cause progressive cholestatic liver disease.
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