| 货号 | 11605-250mg |
| 描述 | 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).1 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.1 Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG.1 Type II D-2-HGA results from gain-of-function mutations in isocitrate dehydrogenase 2, causing it to supplement HOT in converting 2-KG to D-2-HG.2,3 In bacteria, this α-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an α-hydroxyglutaric acid synthetase.4 |
| 别名 | D-2-HG;D-2-Hydroxyglutaric Acid; |
| 供应商 | Cayman |
| 应用文献 | |
| 1.Kranendijk, M.,Struys, E.A.,Salomons, G.S., et al. Progress in understanding 2-hydroxyglutaric acidurias. J.Inherit.Metab.Dis. 35(4), 571-587 (2012). 2.Kranendijk, M.,Struys, E.A.,Van Schaftingen, E., et al. IDH2 Mutations in Patients with D-2-Hydroxyglutaric Aciduria. Science 330(6002), 336 (2014). 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.Reeves, H.C., and Ajl, S.J. Alpha-hydroxyglutaric acid synthetase. Journal of Bacteriology 84, 186-187 (1962). | |
| 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 | 103404-90-6 |
| 稳定性 | ≥ 2 years |
| 本官网所有报价均为常温或者蓝冰运输价格，如有产品需要干冰运输，需另外加收干冰运输费。 |