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alpha-Hydroxyglutaric acid (2-HG) is an alpha-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 (Cay-21123). 2-HG can also be metabolized from isocitrate by mutated forms of isocitrate dehydrogenase 1 (IDH1) and IDH2. 2-HG is structurally similar to alpha-ketoglutarate, the product of wild-type isocitrate dehydrogenases, and competitively inhibits alpha-ketoglutarate-dependent dioxygenases, including histone lysine demethylases and DNA hydroxylases.Formal Name: 2-hydroxy-pentanedioic acid. CAS Number: 2889-31-8. Synonyms: 2-HG, 2-Hydroxyglutaric Acid. Molecular Formula: C5H8O5. Formula Weight: 148.1. Purity: >95%. Formulation: (Request formulation change), A solution in ethanol. Solubility: DMF: 30 mg/ml, DMSO: 30 mg/ml, Ethanol: 30 mg/ml, PBS (pH 7.2): 10 mg/ml. SMILES: OC(C(CCC(O)=O)O)=O. InChi Code: InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10). InChi Key: HWXBTNAVRSUOJR-UHFFFAOYSA-N.
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