L-2-hydroxyglutarate dehydrogenase
Template:Short description Template:Infobox enzyme In enzymology, an L-2-hydroxyglutarate dehydrogenase (EC 1.1.99.2) 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. [1][2] Enzymes which preferentially catalyze the conversion of the (R) stereoisomer of 2-oxoglutarate also exist in both mammals and plants [3] [4] 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:
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- (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 [1] [2] [5]
See also
- L2HGDH
- D2HGDH
- D-2-hydroxyglutarate dehydrogenase
- 2-hydroxyglutarate synthase
- Alpha-Hydroxyglutaric acid
- 2-Hydroxyglutaric aciduria
- Hydroxyacid-oxoacid transhydrogenase
References
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Further reading
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