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D-α-Hydroxyglutaric Acid
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
D-α-Hydroxyglutaric Acid图片
CAS NO:13095-47-1
规格:98%
分子量:148.1
包装与价格:
包装价格(元)
10mg电议
25mg电议
50mg电议

产品介绍
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).
CAS:13095-47-1
分子式:C5H8O5
分子量:148.1
纯度:98%
存储:Store at -20°C

Background:

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.