In enzymology, an L-2-hydroxyglutarate dehydrogenase () is an enzyme that catalyzes the chemical reaction (S)-2-hydroxyglutarate + acceptor 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 See also L2HGDH D2HGDH D-2-hydroxyglutarate dehydrogenase 2-hydroxyglutarate synthase Alpha-Hydroxyglutaric acid 2-Hydroxyglutaric aciduria Hydroxyacid-oxoacid transhydrogenase References Further reading EC 1.1.99 Enzymes of unknown structure