Isovaleryl-CoA Dehydrogenase

Synonyms

CoA Dehydrogenase, Isovaleryl

Dehydrogenase, Isovaleryl CoA

Dehydrogenase, Isovaleryl-CoA

Dehydrogenase, Isovaleryl-Coenzyme A

Isovaleryl CoA Dehydrogenase

Isovaleryl Coenzyme A Dehydrogenase

Isovaleryl-Coenzyme A Dehydrogenase

A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects in the enzyme, is associated with isovaleric acidemia (IVA).