D-alpha-Hydroxyglutaric Acid

D-alpha-Hydroxyglutaric acid (D-2-HG) is an alpha-hydroxy acid that is over-produced in the human neurometabolic disease D-2-hydroxyglutaric aciduria (D-2-HGA). It is normally synthesized from 2-ketoglutarate (2-KG) by hydroxyacid-oxoacid transhydrogenase (HOT), although defects in HOT are not known to be associated with D-2-HGA. Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG. Type II D-2-HGA results from gain-of-function mutations in isocitrate dehydrogenase 2 (IDH2), causing it to supplement HOT in converting 2-KG to D-2-HG. In bacteria, this alpha-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an alpha-hydroxyglutaric acid synthetase.Formal Name: 2R-hydroxy-pentanedioic acid. CAS Number: 13095-47-1. Synonyms: D-2-HG, D-2-Hydroxyglutaric Acid. Molecular Formula: C5H8O5. Formula Weight: 148.1. Purity: >95%. Formulation: (Request formulation change), A solution in ethanol. Solubility: DMF: 30 mg/ml, DMSO: 30 mg/ml, Ethanol: 30 mg/ml, PBS (pH 7.2): 10 mg/ml. lambdamax: 212 nm. SMILES: OC([C@@H](CCC(O)=O)O)=O. InChi Code: InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)/t3-/m1/s1. InChi Key: HWXBTNAVRSUOJR-GSVOUGTGSA-N.