L-2-hydroxyglutarate dehydrogenase

In enzymology, an L-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. Enzymes which preferentially catalyze the conversion of the (R) stereoisomer of 2-oxoglutarate also exist in both mammals and plants and are named D-2-hydroxyglutarate dehydrogenase. L-2-hydroxyglutarate is produced by promiscuous action of malate dehydrogenase on 2-oxoglutarate; L-2-hydroxyglutarate dehydrogenase is an example of a metabolite repair enzyme that oxidizes L-2-hydroxyglutarate back to 2-oxoglutarate.

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 (L2HGDH) or in the model plant Arabidopsis thaliana (At3g56840) leads to accumulation of L-2-hydroxyglutarate. In humans this results in the fatal neurometabolic disorder 2-Hydroxyglutaric aciduria whereas plants seem to be unaffected by elevated cellular concentrations of this compound