Clinical Significance
Deficiency in this enzyme in humans (D2HGDH) or in the model plant Arabidopsis thaliana 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.
Read more about this topic: 2-hydroxyglutarate Dehydrogenase
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