D-2-hydroxyglutarate dehydrogenase


In enzymology, D-2-hydroxyglutarate dehydrogenase is an enzyme that catalyzes the chemical reaction
The two substrates of this enzyme are -2-hydroxyglutaric acid and an electron acceptor. Its products are α-ketoglutaric acid and the corresponding 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 -2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:
  • -2-hydroxyglutarate: 2-oxidoreductase
  • alpha-hydroxyglutarate dehydrogenase
  • alpha-hydroxyglutarate dehydrogenase
  • 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 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.