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.
References in periodicals archive ?
Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia.
It was found that the intensity of acylcarnitine peaks significantly increased at acetonitrile concentration of 90% and above, but this resulted in peaks appearing in acylcarnitine profiles, specifically at m/z 302, this can result in a misinterpretation of the blood acylcarnitine profile as the same ion with an m/z of 302 appears as isovaleryl-/valerylcarnitine parent ion, which is increased in patients suffering of isovaleric acidemia (OMIM 243500) an autosomal recessive disorder of leucine catabolism caused by reduced activity of isovaleryl-CoA dehydrogenase (18).
organic acidurias such as glutaryl-CoA dehydrogenase, 3-hydroxy-3-methylglutaryl-CoA lyase, [beta]-ketothiolase, Propionyl-CoA carboxylase, Methylmalonyl-CoA mutase, and Isovaleryl-CoA dehydrogenase) (Vreken et al., 1999).
Isovaleric acidemia (IVA) is an autosomal recessive genetic disorder of the enzyme isovaleryl-CoA dehydrogenase, which is involved in leucine metabolism (1).