| 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.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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