D-2-hydroxyglutarate dehydrogenase
Template:Short description Template:Enzyme In enzymology, a D-2-hydroxyglutarate dehydrogenase (Template:EC number) is an enzyme that catalyzes the chemical reaction
- (R)-2-hydroxyglutarate + acceptor 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:
- (R)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
- alpha-hydroxyglutarate dehydrogenase
- alpha-hydroxyglutarate dehydrogenase (NADTemplate:+ specific)
- alpha-hydroxyglutarate oxidoreductase
- alpha-ketoglutarate reductase
- hydroxyglutaric dehydrogenase
- D-alpha-hydroxyglutarate dehydrogenase
- D-alpha-hydroxyglutarate:NADTemplate:+ 2-oxidoreductase
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
- D2HGDH
- L2HGDH
- L-2-hydroxyglutarate dehydrogenase
- 2-hydroxyglutarate synthase
- Alpha-Hydroxyglutaric acid
- 2-Hydroxyglutaric aciduria
- Hydroxyacid-oxoacid transhydrogenase
References
Further reading
Template:Alcohol oxidoreductases Template:Enzymes Template:Portal bar