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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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蛋白别名: Butyryl-CoA dehydrogenase; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase, mitochondrial; IVD
基因别名: ACAD2; IVD
UniProt ID: (Human) P26440, (Rat) P12007
Entrez Gene ID: (Human) 3712, (Dog) 100856316, (Rat) 24513