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. 3-Hydroxyglutaric acid is one of several metabolites produced when insufficient levels of GCDH are available. 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. 3-Hydroxyglutaric acid is used as a biomarker of GCDH deficiency.
