Formulation: A crystalline solid
Formal Name: 2-hydroxy-pentanedioic acid, disodium salt
Purity: ≥95%
Formula Markup: C5H6O5 • 2Na
Formula Weight: 192,1
Shelf life (days): 730
CAS Number: 40951-21-1
Notes: α-Hydroxyglutaric acid (2-HG) is an α-hydroxy acid. It is metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases, and mutations in these enzymes lead to 2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by increased levels of L-2-hydroxyglutaric acid (Item No. 21123).{26770,26771,26772} 2-HG can also be metabolized from isocitrate by mutated forms of isocitrate dehydrogenase 1 (IDH1) and IDH2.{26773,26775} 2-HG is structurally similar to α-ketoglutarate, the product of wild-type isocitrate dehydrogenases, and competitively inhibits α-ketoglutarate-dependent dioxygenases, including histone lysine demethylases and DNA hydroxylases.{26775,26774,26769}
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