| 货号 | 16374-10mg |
| 描述 | α-Hydroxyglutaric acid (2-HG) is an α-hydroxy acid. It is normally metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases, and mutations in these enzymes cause 2-hydroxyglutaric aciduria, a neurometabolic disorder.1,2,3 Recent studies have found that mutations in isocitrate dehydrogenases 1 and 2, typically associated with certain cancers, can cause these enzymes to convert isocitrate to 2-HG, rather than α-ketoglutarate.4,5 2-HG is structurally similar to α-ketoglutarate and competitively inhibits α-ketoglutarate-dependent dioxygenases, including lysine demethylases and DNA hydroxylases.5,6,7 |
| 别名 | 2-HG;2-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.Struys, E.A.,Salomons, G.S.,Achouri, Y., et al. Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause D-2-hydroxyglutaric aciduria. American Journal of Human Genetics 76, 358-360 (2005). 4.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). 5.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). 6.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). 7.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 | 192.1 |
| 分子式 | C5H6O5 • 2Na |
| CAS号 | 40951-21-1 |
| 稳定性 | ≥ 2 years |
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