D-2-hydroxyglutarate dehydrogenase

In enzymology, a D-2-hydroxyglutarate dehydrogenase (EC 1.1.99.39) is an enzyme that catalyzes the chemical reaction In enzymology, a D-2-hydroxyglutarate dehydrogenase (EC 1.1.99.39) is an enzyme that catalyzes the chemical reaction 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. 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.