产品描述:
Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial matrix protein that catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide in the catabolic pathways of lysine, hydroxylysine, and tryptophan metabolism.{13906} 3-Hydroxyglutaric acid is one of several metabolites produced when insufficient levels of GCDH are available.{16334,13906} Urinary levels of 3-hydroxyglutaric acid are elevated during glutaric acidemia type 1, an autosomal recessive GCDH deficiency disorder that can lead to neurodegeneration if left untreated.{16334,13906} 3-Hydroxyglutaric acid is used as a biomarker of GCDH deficiency.{16334,13906}