Английская Википедия:D-2-hydroxyglutarate dehydrogenase

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Шаблон:Enzyme In enzymology, a D-2-hydroxyglutarate dehydrogenase (Шаблон:EC number) is an enzyme that catalyzes the chemical reaction

(R)-2-hydroxyglutarate + acceptor <math>\rightleftharpoons</math> 2-oxoglutarate + reduced acceptor

Thus, the two substrates of this enzyme are (R)-2-hydroxyglutarate and acceptor, whereas its two products are 2-oxoglutarate and reduced acceptor.

The enzyme activity has been confirmed in animals[1] as well as in plants .[2]

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 (R)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:

Шаблон:Div col

  • (R)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
  • alpha-hydroxyglutarate dehydrogenase
  • alpha-hydroxyglutarate dehydrogenase (NADШаблон:+ specific)
  • alpha-hydroxyglutarate oxidoreductase
  • alpha-ketoglutarate reductase
  • hydroxyglutaric dehydrogenase
  • D-alpha-hydroxyglutarate dehydrogenase
  • D-alpha-hydroxyglutarate:NADШаблон:+ 2-oxidoreductase

Шаблон:Div col end

Clinical significance

Deficiency in this enzyme in humans (D2HGDH) or in the model plant Arabidopsis thaliana (At4g36400) leads to massive accumulation of D-2-hydroxyglutarate. In humans this results in the fatal neurometabolic disorder 2-Hydroxyglutaric aciduria whereas plants seem to be to a large extent unaffected by high cellular concentrations of this compound.[3][4]

See also

References

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Further reading

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Шаблон:1.1-enzyme-stub