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The condensation product of isovaleric acid and coenzyme A; an intermediate in the catabolism of l-leucine.
References in periodicals archive ?
Defects in isovaleryl-CoA, 2 methylbutyryl-CoA, and hexanoyl-CoA dehydrogenases cause accumulation of their respective CoA esters, which conjugate with glycine to form
Depois que a leucina e metabolizada a KIC, e metabolizado em isovaleryl-CoA pela enzima desidrogenase nas mitocondrias, ou em beta-hidroxi-beta-metilbutirato (HMB) no citosol, pela enzima dioxigenase (Nissen, 1992).
A maioria das KIC e convertida em isovaleryl-CoA redutase, enquanto que em condicoes normais, cerca 5% da oxidacao da leucina acontece via KIC-dioxigenase e convertida em HMB.
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).
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.
Patients with MMA do not have all of the features of isovaleric acidemia, including the characteristic odor of sweaty feet, which is related to isovaleryl-CoA accumulation.
Isovaleric acidemia (IVA) is an autosomal recessive genetic disorder of the enzyme isovaleryl-CoA dehydrogenase, which is involved in leucine metabolism (1).