glutaryl-CoA dehydrogenase

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glu·ta·ryl-CoA de·hy·dro·gen·ase

an enzyme that catalyzes the reaction of glutaryl-CoA with an acceptor to form crotonoyl-CoA, CO2, and the reduced acceptor; a deficiency of this enzyme will lead to either glutaric acidemia type I or hyperoxaluria type II.
Farlex Partner Medical Dictionary © Farlex 2012


A gene on chromosome 19p13.2 that encodes glutaryl-CoA dehydrogenase, a member of the acyl-CoA dehydrogenase family, which catalyses the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2 in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor.

Molecular pathology
Defects in GCDH cause glutaric aciduria type 1.
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References in periodicals archive ?
Guideline for the diagnosis and management of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I).
Late-onset neurologic disease in glutaryl-CoA dehydrogenase deficiency.
1) is notable for lactic and glutaric acid peaks, secondary to diminished perfusion and defective glutaryl-CoA dehydrogenase activities, respectively.
Glutaric aciduria type 1 (GA-1) is an autosomal recessive disorder caused by deficiency of glutaryl-CoA dehydrogenase, which is the key mitochondrial enzyme involved in the final degradation of lysine, L-hydroxylysine and L-tryphtophan.
Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiency.
Teaching NeuroImages: Glutaric aciduria type 1 (glutaryl-CoA dehydrogenase deficiency).
Glutaric aciduria type 1 is the most common OA in South Africa and is caused by AR deficiency of glutaryl-CoA dehydrogenase (GCDH), a critical enzyme in the infantile neuronal lysine catabolic pathway.
(1.) van der Watt G, Owen EP, Berman P, et al.Glutaric aciduria type 1 in South Africa-high incidence of glutaryl-CoA dehydrogenase deficiency in black South Africans.
Assignment of human glutaryl-CoA dehydrogenase gene (GCDH) to the short arm of chromosome 19 (19p13.2) by in situ hybridization and somatic cell hybrid analysis.
Screening the urine for a deficiency of glutaryl-CoA dehydrogenase makes the diagnosis.