Cloned (Comment) | Organism |
---|---|
expressed in HEK-293T cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
DELTA-exon9 | the mutation leads to severely reduced enzyme activity and is associated with L-2-hydroxyglutaric aciduria | Homo sapiens |
E176D | the mutation leads to severely reduced enzyme activity and is associated with L-2-hydroxyglutaric aciduria | Homo sapiens |
K81E | the mutation leads to severely reduced enzyme activity and is associated with L-2-hydroxyglutaric aciduria | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
D-2-hydroxyglutarate | - |
Homo sapiens | |
additional information | the activity is not affected by 0.01 mM Co2+, 0.01 mM Zn2+, or 1 mM EDTA | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.8 | - |
L-2-hydroxyglutarate | in the absence of FAD, pH and temperature not specified in the publication | 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 | - |
- |
- |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-2-hydroxyglutarate + a quinone | - |
Homo sapiens | 2-oxoglutarate + a quinol | - |
? |
Subunits | Comment | Organism |
---|---|---|
? | x * 45000, SDS-PAGE | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
L-2-hydroxyglutarate dehydrogenase | - |
Homo sapiens |
lhgO | - |
Homo sapiens |
YgaF | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
FAD | dependent on | Homo sapiens | |
additional information | not stimulated by FMN | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | enzyme mutations cause L-2-hydroxyglutaric aciduria | Homo sapiens |