isovaleryl-CoA dehydrogenase

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i·so·va·ler·yl-CoA de·hy·dro·gen·ase

an enzyme that participates in the catabolism of l-leucine; it converts isovaleryl-CoA to 3-methylcrotonyl-CoA using FAD; a deficiency in this enzyme will result in isovaleric acidemia.


A gene on chromosome 15q14-q15 that encodes isovaleryl-CoA dehydrogenase, a mitochondrial matrix enzyme that catalyses the third step in leucine catabolism.

Molecular pathology
Defects in IVD leads to a toxic accumulation of isovaleric acid in the brain or isovaleric acidaemia.
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