BCKDH E1-alpha The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Belongs to the BCKDHA family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.2.4.4; Lyase; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 7|7 A3
Cellular Component:  mitochondrial alpha-ketoglutarate dehydrogenase complex
Molecular Function:  3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; alpha-ketoacid dehydrogenase activity; metal ion binding; oxidoreductase activity; oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor; protein-containing complex binding
Biological Process:  branched-chain amino acid catabolic process
Reference #:  P50136 (UniProtKB)
Alt. Names/Synonyms: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKAD E1[a]; BCKDE1A; BCKDH E1-alpha; Bckdha; Branched chain ketoacid dehydrogenase E1, alpha polypeptide; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
Gene Symbols: Bckdha
Molecular weight: 50,371 Da
Basal Isoelectric point: 8.14  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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BCKDH E1-alpha

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein