" /> 3-Methyl-2-Oxobutanoate dehydrogenase (Lipoamide) - CISMeF





Preferred Label : 3-Methyl-2-Oxobutanoate dehydrogenase (Lipoamide);

MeSH definition : 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.;

MeSH synonym : oxo-acid dehydrogenase, branched-chain; branched chain alpha keto acid decarboxylase; branched chain ketoacid dehydrogenase; 2 oxoisocaproate dehydrogenase; 2-oxoisovalerate dehydrogenase (lipoamide); branched chain 2 oxo acid dehydrogenase; branched-chain oxo-acid dehydrogenase; 2-Oxoisocaproate Dehydrogenase; dehydrogenase, 2-oxoisocaproate; branched-chain keto acid dehydrogenase; branched chain keto acid dehydrogenase; bcka decarboxylase; decarboxylase, bcka; branched chain alpha-keto acid decarboxylase; branched-chain 2-oxo acid dehydrogenase; branched chain oxo acid dehydrogenase; dehydrogenase, branched-chain oxo-acid; alpha-keto acid dehydrogenase; acid dehydrogenase, alpha-keto; alpha keto acid dehydrogenase; dehydrogenase, alpha-keto acid;

MeSH CAS label : 2-oxoisocaproate dehydrogenase;

MeSH Related Number : 9082-72-8 (CRN);

Registry Number MeSH : EC 1.2.4.4;

Is substance : O;

UNII : EC 1.2.4.4;

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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.

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25/05/2024


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