D-2-hydroxyglutarate dehydrogenase

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. ==See also==