基因名:
GCDH
产品别名:
ACAD5; GCD; GCDH; glutaryl-CoA dehydrogenase; glutaryl-CoA dehydrogenase; glutaryl-CoA dehydrogenase, mitochondrial; glutaryl-Coenzyme A dehydrogenase; 戊二酰辅酶A脱氢酶; 戊二酰辅酶A脱氢酶(GCDH);
背景信息:
Glutaryl-CoA Dehydrogenase Mitochondrial (GCDH) is an enzyme that acts upon glutaryl-coenzyme A, creating crotonyl-coenzyme A. It plays a role in the metabolism of lysine, hydroxylysine and tryptophan. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive Glutaryl-CoA and electron-transfer flavoprotein to (E)-but-2-enoyl-CoA, CO2 and reduced electron-transfer flavoprotein. A defect in this enzyme is associated with neurological condition glutaric acidemia type 1 and cause a progressive form of early-onset generalized dystonia.