Definition: 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).
Other names Isovaleryl Coenzyme A Dehydrogenase; Dehydrogenase, Isovaleryl-Coenzyme A; Dehydrogenase, Isovaleryl-CoA; Dehydrogenase, Isovaleryl CoA; CoA Dehydrogenase, Isovaleryl; A Dehydrogenase, Isovaleryl-Coenzyme; Isovaleryl-Coenzyme A Dehydrogenase; Isovaleryl CoA Dehydrogenase