Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia.
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Protein Aliases: Butyryl-CoA dehydrogenase; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase, mitochondrial; IVD
Gene Aliases: ACAD2; IVD
Molecular Function: dehydrogenase