Isovaleryl Coenzyme A Dehydrogenase (IVD)

Isovaleryl coenzyme A dehydrogenase is an enzyme which catalyzes the conversion of isovaleryl-coenzyme A to methylcrotonyl-CoA. A deficiency of this enzyme causes isovaleric acidemia.In size, IVD precursor and mature proteins produced by class I mutants are indistinguishable from their normal counterparts. Class II, III, and IV mutants make IVD precursor proteins 42 kD in size rather than the normal 45 kD.The deduced 423-amino acid protein shares 35.8% and 31.6% sequence identity with human short and medium chain acyl-CoA dehydrogenases, respectively, and 89.6% sequence identity with rat IVD. The leader sequence was determined to be overall positively charged, as is typical of other nuclear-encoded mitochondrial matrix enzymes. The molecular mass of the mature protein is 43 kD.