In enzymology, D-2-hydroxyglutarate dehydrogenase () is an enzyme that catalyzes the chemical reaction
The two substrates of this enzyme are (R)-2-hydroxyglutaric acid and an electron acceptor. Its products are ñ-ketoglutaric acid and the corresponding reduced acceptor.
The enzyme activity has been confirmed in animals as well as in plants.
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:
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.