BCKDHB polyclonal antibody
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More Files
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Specification
Product Description
Rabbit polyclonal antibody raised against recombinant BCKDHB.
Immunogen
Recombinant protein corresponding to amino acids of human BCKDHB.
Sequence
QVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRD
Host
Rabbit
Reactivity
Human
Form
Liquid
Purification
Antigen affinity purification
Isotype
IgG
Recommend Usage
Immunohistochemistry (1:20-1:50)
Western Blot (1:250-1:500)
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 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
Western Blot (Transfected lysate)
Western blot analysis of Lane 1: Negative control (vector only transfected HEK293T lysate), Lane 2: Over-expression Lysate (Co-expressed with a C-terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells) with BCKDHB polyclonal antibody (Cat # PAB22782).Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining of human stomach with BCKDHB polyclonal antibody (Cat # PAB22782) shows strong cytoplasmic positivity (granular pattern) in glandular cells. -
Gene Info — BCKDHB
Entrez GeneID
594Protein Accession#
P21953Gene Name
BCKDHB
Gene Alias
E1B, FLJ17880, dJ279A18.1
Gene Description
branched chain keto acid dehydrogenase E1, beta polypeptide
Gene Ontology
HyperlinkGene Summary
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq
Other Designations
2-oxoisovalerate dehydrogenase beta subunit|E1b-beta subunit of the branched-chain complex|OTTHUMP00000018048|OTTHUMP00000018049|branched chain alpha-ketoacid dehydrogenase E1-beta subunit|branched chain keto acid dehydrogenase E1 beta polypeptide|branche
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Interactome
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Disease
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