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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.
GCDHA 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.
Defects in GCDH cause glutaric aciduria type 1.