D-2-HYDROXYPENTANEDIOIC ACID DISODIUM SALT

Description

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, 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.

Chemical properties

Pale Orange Solid

The Uses of D-2-HYDROXYPENTANEDIOIC ACID DISODIUM SALT

A potential inhibitor of glutamate carboxypeptidase.

What are the applications of Application

D-α-Hydroxyglutaric acid disodium salt is an α hydroxy acid and the product of the hydrolysis of (R)-5-oxo-2-tetrahydrofurancarboxylic acid.

Biochem/physiol Actions

Biomarker for inborn errors of metabolism and cancer

Storage

Store at -20°C