D-α-Hydroxyglutaric Acid ((R)-2-Hydroxyglutarate) 是神经代谢疾病 D-2-羟基戊二酸尿症中积累的主要代谢物。
| Cas No. | 13095-47-1 |
| 别名 | R-2-羟基戊二酸,(R)-2-Hydroxyglutarate; (R)-2-Hydroxyglutaric acid; (R)-2-Hydroxypentanedioic acid |
| 化学名 | 2R-hydroxy-pentanedioic acid |
| Canonical SMILES | OC([C@@H](CCC(O)=O)O)=O |
| 分子式 | C5H8O5 |
| 分子量 | 148.1 |
| 溶解度 | 75mg/mL in Water (Need ultrasonic); Insoluble in DMSO |
| 储存条件 | 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. |
| Shipping Condition | Evaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request |
| 产品描述 | 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). 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. Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG. Type II D-2-HGA results from gain-of-function mutations in isocitrate dehydrogenase 2 (IDH2), causing it to supplement HOT in converting 2-KG to D-2-HG. In bacteria, this α-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an α-hydroxyglutaric acid synthetase. |
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