| D-2-hydroxyglutarate dehydrogenase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC no. | 1.1.99.39 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| |||||||||
In enzymology, D-2-hydroxyglutarate dehydrogenase (EC 1.1.99.39) is an enzyme that catalyzes the chemical reaction
The two substrates of this enzyme are (R)-2-hydroxyglutaric acid and an electron acceptor. Its products are α-ketoglutaric acid and the corresponding reduced acceptor. [1]
The enzyme activity has been confirmed in animals [2] as well as in plants. [3]
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:
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. [4] [5]