D-alpha-Hydroxyglutaric Acid (sodium salt)

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, 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, disodium salt. CAS Number: 103404-90-6. Synonyms: D-2-HG, D-2-Hydroxyglutaric Acid. Molecular Formula: C5H6O5 . 2Na. Formula Weight: 192.1. Purity: >95%. Formulation: (Request formulation change), A crystalline solid. Solubility: PBS (pH 7.2): 10 mg/ml. SMILES: [O-]C(CC[C@@H](O)C([O-])=O)=O.[Na+].[Na+]. InChi Code: InChI=1S/C5H8O5.2Na/c6-3(5(9)10)1-2-4(7)8,,/h3,6H,1-2H2,(H,7,8)(H,9,10),,/q,2*+1/p-2/t3-,,/m1../s1. InChi Key: DZHFTEDSQFPDPP-HWYNEVGZSA-L.