D-2-hydroxyglutarate dehydrogenase

In enzymology, a D-2-hydroxyglutarate dehydrogenase is an enzyme that catalyzes the chemical reaction


 * (R)-2-hydroxyglutarate + acceptor $$\rightleftharpoons$$ 2-oxoglutarate + reduced acceptor

Thus, the two substrates of this enzyme are (R)-2-hydroxyglutarate and acceptor, whereas its two products are 2-oxoglutarate and reduced acceptor.

The enzyme activity has been confirmed in animals as well as in plants .

Nomenclature
This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is (R)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:


 * (R)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
 * alpha-hydroxyglutarate dehydrogenase
 * alpha-hydroxyglutarate dehydrogenase (NAD specific)
 * alpha-hydroxyglutarate oxidoreductase
 * alpha-ketoglutarate reductase
 * hydroxyglutaric dehydrogenase
 * D-alpha-hydroxyglutarate dehydrogenase
 * D-alpha-hydroxyglutarate:NAD 2-oxidoreductase

Clinical significance
Deficiency in this enzyme in humans (D2HGDH) or in the model plant Arabidopsis thaliana (At4g36400) leads to massive accumulation of D-2-hydroxyglutarate. In humans this results in the fatal neurometabolic disorder 2-Hydroxyglutaric aciduria whereas plants seem to be to a large extent unaffected by high cellular concentrations of this compound.