IVD


isovaleryl-CoA dehydrogenase


ENTREZID: 3712 | Type: Protein Coding | Map: 15q15.1

OMIM: 607036 | 243500


Summary Entrez
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. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2017]