2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial

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

**Definition:** 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (392 aa, ~43 kDa) is encoded by the human BCKDHB gene. This protein is involved in the catabolism of alpha-ketoacids derived from branched chain amino acids.

**Synonyms:** - 2-Oxoisovalerate Dehydrogenase Subunit Beta - BCKDE1B - BCKDH E1-Beta - BCKDHB - Branched Chain Keto Acid Dehydrogenase E1 Subunit Beta - Branched-Chain Beta-Keto Acid Dehydrogenase E1 Component Beta Chain - EC 1.2.4.4 - Testis Secretory Sperm-Binding Protein Li 240mP