3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
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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.
Synonyms: Dehydrogenase, Alpha-Keto Acid, Branched-Chain Keto Acid Dehydrogenase, 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide), 2-Oxoisovalerate Dehydrogenase (Lipoamide), Dehydrogenase, 2-Oxoisocaproate, Dehydrogenase, Branched-Chain Oxo-Acid, 2 Oxoisocaproate Dehydrogenase, Branched-Chain Oxo-Acid Dehydrogenase, Branched Chain Alpha Keto Acid Decarboxylase, 2-oxoisocaproate dehydrogenase, Branched Chain 2 Oxo Acid Dehydrogenase, Oxo-Acid Dehydrogenase, Branched-Chain, Branched Chain Oxo Acid Dehydrogenase, Alpha-Keto Acid Dehydrogenase, 2-Oxoisocaproate Dehydrogenase, Branched-Chain 2-Oxo Acid Dehydrogenase, Branched Chain Keto Acid Dehydrogenase, Branched Chain Alpha-Keto Acid Decarboxylase, BCKA Decarboxylase, Acid Dehydrogenase, Alpha-Keto, Branched Chain Ketoacid Dehydrogenase, Alpha Keto Acid Dehydrogenase, Decarboxylase, BCKA, EC 1.2.4.4
Instance information
2004; 2-OXOISOVALERATE DEHYDROGENASE (LIPOAMIDE) (now 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE)) was indexed under KETONE OXIDOREDUCTASES & MULTIENZYME COMPLEXES 1982-2003, under AMINO ACIDS, BRANCH-CHAIN 1979-1981, under KETO ACIDS 1973-1981, under CARBOXY-LYASES 1974-1979 ,2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
D042942