isovaleryl-CoA dehydrogenase

isovaleryl-CoA dehydrogenase
iso·val·er·yl-CoA de·hy·dro·gen·ase (i″so-və-lerґəl-ko-aґ de-hiґdro-jən-ās) [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.

Medical dictionary. 2011.

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