Protein Variants | Comment | Organism |
---|---|---|
DELTA-exon9 | the mutation is associated with L-2-hydroxyglutaric aciduria | Homo sapiens |
E176D | the mutation is associated with L-2-hydroxyglutaric aciduria | Homo sapiens |
K71E | the mutation is associated with L-2-hydroxyglutaric aciduria | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
D-2-hydroxyglutarate | - |
Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.15 | - |
iodonitrotetrazolium | at pH 7.1 and 30°C | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrial membrane | - |
Homo sapiens | 31966 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-2-hydroxyglutarate + a quinone | Homo sapiens | - |
2-oxoglutarate + a quinol | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
DEAE Sepharose column chromatography and Q-Sepharose column chromatography | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
heart | - |
Homo sapiens | - |
kidney | high expression | Homo sapiens | - |
liver | highest expression | Homo sapiens | - |
skeletal muscle | - |
Homo sapiens | - |
spleen | lowest expression | Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-2-hydroxyglutarate + a quinone | - |
Homo sapiens | 2-oxoglutarate + a quinol | - |
? | |
L-2-hydroxyglutarate + iodonitrotetrazolium | - |
Homo sapiens | 2-oxoglutarate + reduced iodonitrotetrazolium | - |
? |
Synonyms | Comment | Organism |
---|---|---|
L-2-hydroxyglutarate dehydrogenase | - |
Homo sapiens |
L2HGDH | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
FAD | dependent on | Homo sapiens | |
additional information | the enzyme is not stimulated by FMN | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | enzyme mutations cause L-2-hydroxyglutaric aciduria | Homo sapiens |