L-2-hydroxyglutarate dehydrogenase

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Template:Short description Template:Infobox enzyme In enzymology, an L-2-hydroxyglutarate dehydrogenase (Template:EC number) 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 (NADTemplate:+ specific)
  • alpha-hydroxyglutarate oxidoreductase
  • alpha-ketoglutarate reductase
  • hydroxyglutaric dehydrogenase
  • L-alpha-hydroxyglutarate dehydrogenase
  • L-alpha-hydroxyglutarate:NADTemplate:+ 2-oxidoreductase

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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

References

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Further reading

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  1. ↑ 1.0 1.1 Template:Cite journal
  2. ↑ 2.0 2.1 Template:Cite journal
  3. ↑ Template:Cite journal
  4. ↑ Template:Cite journal
  5. ↑ Template:Cite journal
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