EC number 1.1.99.39 BRENDA BRENDA entry KEGG KEGG entry | IntEnz IntEnz view ExPASy NiceZyme view MetaCyc | |
In enzymology, a D-2-hydroxyglutarate dehydrogenase (EC 1.1.99.39) is an enzyme that catalyzes the chemical reaction
Contents
(R)-2-hydroxyglutarate + acceptorThus, 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 as well as in plants .
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:
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.