| isovaleryl-CoA | The condensation product of isovaleric acid and coenzyme A; an intermediate in the catabolism of l-leucine. Synonym: isovalerylcoenzyme A. Isovaleryl-CoA dehydrogenase, an enzyme that participates in the catabolism of l-leucine; it converts isovaleryl-CoA to 3-methylcrotonyl-CoA using FAD; a deficiency in this enzyme will result in isovaleric acidemia. (05 Mar 2000) |
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Synonyms : Isovaleryl CoA Dehydrogenase, Isovaleryl-Coenzyme A Dehydrogenase, A Dehydrogenase, Isovaleryl-Coenzyme, CoA Dehydrogenase, Isovaleryl, Dehydrogenase, Isovaleryl CoA, Dehydrogenase, Isovaleryl-CoA, Dehydrogenase, Isovaleryl-Coenzyme A
| isovaleryl-CoA dehydrogenase |
[EC 1.3.99.10] an enzyme of the oxidoreductase class that catalyzes the dehydrogenation of isovaleryl CoA to 3-methylcrotonyl CoA, using ubiquinone as an electron acceptor. The reaction is a step in the use of leucine as a fuel. Deficiency of the enzyme, an autosomal recessive trait, results in isovalericacidemia.
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