Formulation: A crystalline solid
Formal Name: 2S-hydroxy-pentanedioic acid, disodium salt
Purity: ≥95%
Formula Markup: C5H8O5 • 2Na
Formula Weight: 194,1
Shelf life (days): 1460
CAS Number: 63512-50-5
Notes: L-α-Hydroxyglutaric acid (L-2-HG) is an α-hydroxy acid. It is metabolized to 2-oxoglutarate (α-ketoglutarate) by L-2-hydroxyglutarate dehydrogenase, and mutations in this enzyme lead to 2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by increased L-2-HG levels.{26770,26771} L-2-HG is structurally similar to α-ketoglutarate and competitively inhibits α-ketoglutarate-dependent dioxygenases, including several involved in histone lysine and DNA demethylation.{26769,26774}