3-Hydroxyglutaric Acid

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.Formal Name: 3-hydroxy-pentanedioic acid. CAS Number: 638-18-6. Synonyms: 2,4-Dideoxypentaric Acid, FA 5:1,O3, 3-HG, 3-Hydroxyglutarate, beta-Hydroxyglutaric Acid, 3-Hydroxypentanedioic Acid, 3-OH-GA. Molecular Formula: C5H8O5. Formula Weight: 148.1. Purity: >95%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 30 mg/ml, DMSO: 30 mg/ml, Ethanol: 30 mg/ml, PBS (pH 7.2): 10 mg/ml. SMILES: OC(CC(O)CC(O)=O)=O. InChi Code: InChI=1S/C5H8O5/c6-3(1-4(7)8)2-5(9)10/h3,6H,1-2H2,(H,7,8)(H,9,10). InChi Key: ZQHYXNSQOIDNTL-UHFFFAOYSA-N.