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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.
Isovaleryl-CoA dehydrogenase activity in isovaleric acidemia fibroblasts using an improved tritium release assay.
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).