Cloned (Comment) | Organism |
---|---|
expression in Escherichia coli | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
I147S | D-2-hydroxyglutaric aciduria disease-related variant. The cofactor binding site is compromised by the single amino acid replacement. The variant forms aggregates that are unable to bind the FAD cofactor | Homo sapiens |
V444A | D-2-hydroxyglutaric aciduria disease-related variant. The cofactor binding site is compromised by the single amino acid replacement. The variant forms aggregates that are unable to bind the FAD cofactor | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
(R)-2-hydroxyglutarate + electron transfer flavoprotein | Homo sapiens | - |
2-oxoglutarate + reduced electron transfer flavoprotein | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q8N465 | - |
- |
Purification (Comment) | Organism |
---|---|
- |
Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
(R)-2-hydroxyglutarate + electron transfer flavoprotein | - |
Homo sapiens | 2-oxoglutarate + reduced electron transfer flavoprotein | - |
? |
Synonyms | Comment | Organism |
---|---|---|
D2HGDH | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
FAD | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
physiological function | the enzyme directly reduces recombinant human electron transferring flavoprotein (ETF), thus establishing a metabolic link between the oxidation of D-2-hydroxyglutarate and the mitochondrial electron transport chain | Homo sapiens |