Entrez Summary: This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. [provided by RefSeq, Jul 2008].
UniProt Summary: Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate. {ECO:0000269|PubMed:15070399}.
Pfam DomainsGO Terms
Pfam Domains
FAD binding 4
FAD-oxidase C
GO Terms
(R)-2-hydroxyglutarate dehydrogenase activity
lactate oxidation
D-lactate dehydrogenase (cytochrome) activity
response to cobalt ion
lactate metabolic process
2-oxoglutarate metabolic process
response to manganese ion
response to magnesium ion
FAD binding
response to zinc ion
extrinsic component of cytoplasmic side of plasma membrane
dicarboxylic acid metabolic process
respiratory electron transport chain
response to calcium ion
cellular respiration
electron transport chain
energy derivation by oxidation of organic compounds
mitochondrial matrix
response to metal ion
generation of precursor metabolites and energy
cofactor metabolic process
organic hydroxy compound metabolic process
monocarboxylic acid metabolic process
response to inorganic substance
carboxylic acid metabolic process
oxidation-reduction process
oxoacid metabolic process
organic acid metabolic process
mitochondrion
small molecule metabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)
Compound Hit
No hits were found.
Most Correlated Genes in Chemogenomics
No correlation found to any other genes in chemogenomics.
Tissues where Essential in the Avana Dataset (DepMap 20Q1)
Global Fraction of Cell Lines Where Essential: 0/739