Isovaleryl-CoA Dehydrogenase

**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).

**Tree numbers:** - D08.811.682.660.462

**Synonyms:** - Isovaleryl CoA Dehydrogenase - Isovaleryl-Coenzyme A Dehydrogenase