3-metyl-2-oxobutanoatdehydrogenas (Lipoamid) (3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide))

3-metyl-2-oxobutanoatdehydrogenas (Lipoamid)

Ett ketonoxidoreduktas som katalyserar den totala omvandlingen av alfa-keto-syror till acyl-CoA och CO2. Enzymet kräver TIAMINDIFOSFAT som en kofaktor. Defekter i gener som kodar för subenheter av enzymet är en orsak till MAPLE SIRAP URINSJUKDOM. Enzymet klassificerades tidigare som EC 1.2.4.3.

Förklaring på engelska

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.

Synonym, svenska:

Synonym, engelska: 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