A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
Inga svenska synonymer finns.
2-Oxoisocaproate Dehydrogenase — 2 Oxoisocaproate Dehydrogenase — Dehydrogenase, 2-Oxoisocaproate — 2-Oxoisovalerate Dehydrogenase (Lipoamide) — Branched-Chain Keto Acid Dehydrogenase — Branched Chain Keto Acid Dehydrogenase — BCKA Decarboxylase — Decarboxylase, BCKA — Branched Chain Alpha-Keto Acid Decarboxylase — Branched Chain Alpha Keto Acid Decarboxylase — Branched Chain Ketoacid Dehydrogenase — Branched-Chain 2-Oxo Acid Dehydrogenase — Branched Chain 2 Oxo Acid Dehydrogenase — Branched-Chain Oxo-Acid Dehydrogenase — Branched Chain Oxo Acid Dehydrogenase — Dehydrogenase, Branched-Chain Oxo-Acid — Oxo-Acid Dehydrogenase, Branched-Chain — Alpha-Keto Acid Dehydrogenase — Acid Dehydrogenase, Alpha-Keto — Alpha Keto Acid Dehydrogenase — Dehydrogenase, Alpha-Keto Acid