L-2-hydroxyglutarate dehydrogenase
Class of enzymes
From Wikipedia, the free encyclopedia
In enzymology, L-2-hydroxyglutarate dehydrogenase (EC 1.1.99.2) is an enzyme that catalyzes the chemical reaction
-2-hydroxyglutaric_acid.svg)
| L-2-hydroxyglutarate dehydrogenase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC no. | 1.1.99.2 | ||||||||
| CAS no. | 9028-80-2 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
The two substrates of this enzyme are (S)-2-hydroxyglutaric acid and an electron acceptor. Its products are α-ketoglutaric acid and the corresponding reduced acceptor.[1][2][3] Enzymes which preferentially catalyze the conversion of the (R) stereoisomer of 2-oxoglutarate also exist in both mammals and plants[4][5] and are named D-2-hydroxyglutarate dehydrogenase. L-2-hydroxyglutarate is produced by promiscuous action of malate dehydrogenase on 2-oxoglutarate; L-2-hydroxyglutarate dehydrogenase is an example of a metabolite repair enzyme that oxidizes L-2-hydroxyglutarate back to 2-oxoglutarate.
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 (S)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:
- (S)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
- alpha-hydroxyglutarate dehydrogenase
- alpha-hydroxyglutarate dehydrogenase (NAD+ specific)
- alpha-hydroxyglutarate oxidoreductase
- alpha-ketoglutarate reductase
- hydroxyglutaric dehydrogenase
- L-alpha-hydroxyglutarate dehydrogenase
- L-alpha-hydroxyglutarate:NAD+ 2-oxidoreductase
Clinical significance
Deficiency in this enzyme in humans (L2HGDH) or in the model plant Arabidopsis thaliana (At3g56840) leads to accumulation of L-2-hydroxyglutarate. In humans this results in the fatal neurometabolic disorder 2-Hydroxyglutaric aciduria whereas plants seem to be unaffected by elevated cellular concentrations of this compound [2] [3] [6]
