Gene:
isovaleryl Coenzyme A dehydrogenase
Gene ID:
193010
Symbol:
IVD
Species:
1000
Description:
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.