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; Lyase; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 19q13.2
Cellular Component:  mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion
Molecular Function:  3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; metal ion binding; protein binding
Biological Process:  branched-chain amino acid catabolic process; oxidation-reduction process
Disease: Maple Syrup Urine Disease
Reference #:  P12694 (UniProtKB)
Alt. Names/Synonyms: 2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1-alpha; BCKDHA; branched chain keto acid dehydrogenase E1, alpha polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; FLJ45695; MSU; MSUD1; ODBA; OVD1A
Gene Symbols: BCKDHA
Molecular weight: 50,471 Da
Basal Isoelectric point: 8.42  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
Select Structure to View Below

BCKDH E1-alpha

Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene