glutaryl-CoA dehydrogenase

Also found in: Wikipedia.

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.


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.
References in periodicals archive ?
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.
Glutaric aciduria type 1 in South Africa-high incidence of glutaryl-CoA dehydrogenase deficiency in black South Africans.
Neonatal screening for glutaryl-CoA dehydrogenase deficiency.
Assignment of human glutaryl-CoA dehydrogenase gene (GCDH) to the short arm of chromosome 19 (19p13.