2-hydroxyglutarate dehydrogenase

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


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

Thus, the two substrates of this enzyme are (S)-2-hydroxyglutarate and acceptor, whereas its two products are 2-oxoglutarate and reduced acceptor. However, enzymes with specificity to (R)-2-hydroxyglutarate dehydrogenase also exist in both mammals and plants even though they have not been given a unique EC number. This enzyme participates in butanoate metabolism.

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 (S)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:


 * (S)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
 * alpha-hydroxyglutarate dehydrogenase
 * alpha-hydroxyglutarate dehydrogenase (NAD+ specific)
 * alpha-hydroxyglutarate oxidoreductase
 * alpha-ketoglutarate reductase
 * hydroxyglutaric dehydrogenase
 * L-alpha-hydroxyglutarate dehydrogenase
 * L-alpha-hydroxyglutarate:NAD+ 2-oxidoreductase

Clinical significance
Deficiency in this enzyme in humans (D2HGDH) or in the model plant Arabidopsis thaliana 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.