Isovaleryl-CoA Dehydrogenase

Isovaleryl CoA Dehydrogenase; Isovaleryl-Coenzyme A Dehydrogenase; A Dehydrogenase, Isovaleryl-Coenzyme; CoA Dehydrogenase, Isovaleryl; Dehydrogenase, Isovaleryl CoA; Dehydrogenase, Isovaleryl-CoA; Dehydrogenase, Isovaleryl-Coenzyme A
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).