Glutaryl Coenzyme A Dehydrogenase (GCDH)

Glutaryl-CoA dehydrogenase (GCDH) is an acyl dehydrogenase involved in the metabolism of lysine, hydroxylysine, and tryptophan. Specifically, it is responsible for the dehydrogenation and decarboxylation of glutaryl-CoA to crotonyl-CoA in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. The active enzyme exists as a homotetramer in the mitochondrial matrix.  It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Alternatively spliced transcript variants encoding different isoforms have been identified.