产品属性:
产品名称
规格
CAS号
型号
D-α-Hydroxyglutaric Acid
10mg 25mg 50mg
13095-47-1
EY-Y0164557
Cas No.13095-47-1
别名 N/A
化学名 2R-hydroxy-pentanedioic acid
分子式 C5H8O5
分子量 148.1
溶解度 DMF: 30 mg/mL,DMSO: 30 mg/mL,Ethanol: 30 mg/mL,PBS (pH 7.2): 10 mg/mL
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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