|L-α-Hydroxyglutaric Acid Catalog No.GC41478|
Sample solution is provided at 25 µL, 10mM.
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|Cas No.||13095-48-2||SDF||Download SDF|
|Synonyms||2(S)-HG, 2(S)-Hydroxyglutaric Acid, L-2-HG, L-2-Hydroxyglutaric Acid|
|Chemical Name||2S-hydroxy-pentanedioic acid|
|Solubility||10mg/mL in PBS, pH 7.2||Storage||Store at -20°C|
|General tips||For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.Stock solution can be stored below -20℃ for several months.|
|Shipping Condition||Evaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
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.[1 ] 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.
. Rzem, R., Veiga-da-Cunha, M., Noël, G., et al. A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria. Proc. Nat. Acad. Sci. USA 101(48), 16849-16854 (2004).
. Struys, E.A., Verhoeven, N.M., Roos, B., et al. Disease-related metabolites in culture medium of fibroblasts from patients with D-2-hydroxyglutaric aciduria, L-2-hydroxyglutaric aciduria, and combined D/L-2-hydroxyglutaric aciduria. Clin. Chem. 49(7), 1133-1138 (2003).
. Chowdhury, R., Yeoh, K.K., Tian, Y.M., et al. The oncometabolite 2-hydroxyglutarate inhibits histone lysine demethylases. EMBO Rep. 12(5), 463-469 (2011).
. Xu, W., Yang, H., Liu, Y., et al. Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases. Cancer Cell 19(1), 17-30 (2011).