2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial

**Semantic type:** Amino Acid, Peptide, or Protein|Enzyme

**Definition:** 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (445 aa, ~50 kDa) is encoded by the human BCKDHA gene. This protein plays a role in the decarboxylation of alpha-ketoacids derived from branched-chain amino acids.

**Synonyms:** - 2-Oxoisovalerate Dehydrogenase Subunit Alpha - BCKDE1A - BCKDH E1-Alpha - BCKDHA - Branched Chain Keto Acid Dehydrogenase E1 Subunit Alpha - Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Alpha Chain - EC 1.2.4.4