L-2-hydroxyglutarate dehydrogenase


In enzymology, an L-2-hydroxyglutarate dehydrogenase is an enzyme that catalyzes the chemical reaction
Thus, the two substrates of this enzyme are -2-hydroxyglutarate and acceptor, whereas its two products are 2-oxoglutarate and reduced acceptor.
Enzymes which preferentially catalyze the conversion of the 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 -2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:
Deficiency in this enzyme in humans or in the model plant Arabidopsis thaliana 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