BCKDHA polyclonal antibody (A01)
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More Files
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Western Blot (Cell lysate)
BCKDHA polyclonal antibody (A01), Lot # 051212JC01 Western Blot analysis of BCKDHA expression in Y-79 ( Cat # L042V1 ).
Western Blot detection against Immunogen (37 KDa) .
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Specification
Product Description
Mouse polyclonal antibody raised against a partial recombinant BCKDHA.
Immunogen
BCKDHA (NP_000700, 347 a.a. ~ 445 a.a) partial recombinant protein with GST tag.
Sequence
SVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
Host
Mouse
Reactivity
Human
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (37 KDa) .
Storage Buffer
50 % glycerol
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
BCKDHA polyclonal antibody (A01), Lot # 051212JC01 Western Blot analysis of BCKDHA expression in Y-79 ( Cat # L042V1 ).Western Blot (Recombinant protein)
ELISA
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Gene Info — BCKDHA
Entrez GeneID
593GeneBank Accession#
NM_000709Protein Accession#
NP_000700Gene Name
BCKDHA
Gene Alias
BCKDE1A, FLJ45695, MSU, MSUD1, OVD1A
Gene Description
branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Ontology
HyperlinkGene Summary
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene
Other Designations
2-oxoisovalerate dehydrogenase (lipoamide)|branched-chain alpha-keto acid dehydrogenase complex E1 alpha subunit|maple syrup urine disease
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Interactome
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Pathway
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Disease
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