Shelf life (days): 365.0
Formulation: A solution in ethanol
Formal Name: 2R-hydroxy-pentanedioic acid
Purity: ≥95%
Formula Markup: C5H8O5
Formula Weight: 148.11402
CAS Number: 13095-47-1
Notes: D-α-Hydroxyglutaric acid (D-2-HG) is an α-hydroxy acid that is over-produced in the human neurometabolic disease D-2-hydroxyglutaric aciduria (D-2-HGA).{26892} 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.{26892} Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG.{26892} 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.{26891,26772} In bacteria, this α-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an α-hydroxyglutaric acid synthetase.{26893}
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