[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). ( MSH )]
UMLS (CSP) C0064095 - Amino Acid, Peptide, or Protein
- Enzyme