InterPro domain: IPR034183

Isovaleryl-CoA dehydrogenase (IVD) is an acyl-CoA dehydrogenase, which catalyzes the third step in leucine catabolism, the conversion of isovaleryl-CoA (3-methylbutyryl-CoA) into 3-methylcrotonyl-CoA. IVD is a homotetramer and has the greatest affinity for small branched chain substrates [ 1 , 2 ].

1. Molecular basis of isovaleric acidemia and the study of the acyl-CoA dehydrogenase family. Adv Neurol 48, 107-31
2. Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver. J. Biol. Chem. 262, 7982-9