| 货号 | 21123-1mg |
| 描述 | (2S)-Hydroxyglutaric acid is a natural α-hydroxy acid that is normally metabolized to 2-oxoglutarate (α-ketoglutarate) by L-2-hydroxyglutarate dehydrogenase (L2HGDH). Mutations in L2HGDH result in L-2-hydroxyglutaric aciduria, an autosomal recessive disorder resulting in neurological impairment at a young age.1,2 Mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2, typically associated with certain cancers, can cause these enzymes to convert isocitrate to 2-hydroxyglutaric acid, rather than 2-oxoglutarate.3,4 (2S)-Hydroxyglutaric acid inhibits 2-oxoglutarate-dependent dioxygenases, including lysine demethylases and DNA hydroxylases.4,5,6 |
| 别名 | 2(S)-HG;L-2-Hydroxyglutaric Acid;L-α-Hydroxyglutaric Acid; |
| 供应商 | Cayman |
| 应用文献 | |
| 1.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. Proceedings of the National Academy of Sciences of the United States of America 101(48), 16849-16854 (2004). 2.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 combind D/L-2-hydroxyglutaric aciduria. Clinical Chemistry 49(7), 1133-1138 (2003). 3.Ward, P.,Patel, J.,Wise, D.R., et al. The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting α-ketoglutarate to 2-hydroxyglutarate. Cancer Cell 17, 225-234 (2010). 4.Yang, H.,Ye, D.,Guan, K.L., et al. IDH1 and IDH2 mutations in tumorigenesis: Mechanistic insights and clinical perspectives. Clinical Cancer Research 18(20), 5562-5571 (2012). 5.Xu, W.,Yang, H.,Liu, Y., et al. Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases. Cancer Cell 19, 17-30 (2011). 6.Chowdhury, R.,Yeoh, K.K.,Tian, Y.M., et al. The oncometabolite 2-hydroxyglutarate inhibits histone lysine demethylases. EMBO reports 12(5), 463-469 (2011). | |
| 运输条件 | Room temperature in continental US; may vary elsewhere |
| 存放说明 | -20 |
| 纯度 | ≥95% |
| 计算分子量 | 148.1 |
| 分子式 | C5H8O5 |
| CAS号 | 13095-48-2 |
| 稳定性 | ≥ 1 year |
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