IVD

(redirected from isovaleryl-CoA dehydrogenase)
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IVD

(Interactive VideoDisc) See interactive video.
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Isovaleric academia (WA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of isovaleryl-CoA dehydrogenase.
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).
Isovaleric acidemia (IVA) is an autosomal recessive genetic disorder of the enzyme isovaleryl-CoA dehydrogenase, which is involved in leucine metabolism (1).