D-α-Hydroxyglutaric Acid (sodium salt) – 10 mg

Brand:
Cayman
CAS:
103404-90-6
Storage:
-20
UN-No:
Non-Hazardous - /

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).{26892} 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.{26892} Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG.{26892} 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.{26891,26772} In bacteria, this α-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an α-hydroxyglutaric acid synthetase.{26893}  

 

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SKU: 11605 - 10 mg Category:

Description

An α-hydroxy acid that is over-produced in the human neurometabolic disease D-2-hydroxyglutaric aciduria


Formal name: 2R-hydroxy-pentanedioic acid, disodium salt

Synonyms:  D-2-HG|D-2-Hydroxyglutaric Acid

Molecular weight: 192.1

CAS: 103404-90-6

Purity: ≥95%

Formulation: A crystalline solid


Product Type|Biochemicals||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Neuroscience