BCKDHA 293T Cell Transient Overexpression Lysate(Denatured)
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More Files
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Specification
Transfected Cell Line
293T
Plasmid
pCMV-BCKDHA full-length
Host
Human
Theoretical MW (kDa)
49.06
Quality Control Testing
Transient overexpression cell lysate was tested with Anti-BCKDHA antibody (H00000593-B01) by Western Blots.
SDS-PAGE Gel
BCKDHA transfected lysate.
Western Blot
Lane 1: BCKDHA transfected lysate ( 49.06 KDa)
Lane 2: Non-transfected lysate.Storage Buffer
1X Sample Buffer (50 mM Tris-HCl, 2% SDS, 10% glycerol, 300 mM 2-mercaptoethanol, 0.01% Bromophenol blue)
Storage Instruction
Store at -80°C. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot
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Gene Info — BCKDHA
Entrez GeneID
593GeneBank Accession#
NM_000709.2Protein Accession#
NP_000700.1Gene 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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