Protein Variants | Comment | Organism |
---|---|---|
additional information | A320G, G607T, naturally occurring mutations found in L-2-hydroxyglutaric aciduria affected patients | Homo sapiens |
General Stability | Organism |
---|---|
very labile in purification processes | Rattus norvegicus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | bound to membrane | Rattus norvegicus | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
(S)-2-hydroxyglutarate + acceptor | Homo sapiens | enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria | 2-oxoglutarate + reduced acceptor | - |
? | |
(S)-2-hydroxyglutarate + FAD | Rattus norvegicus | enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria | 2-oxoglutarate + FADH2 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q9H9P8 | families affected from L-2-hydroxyglutaric aciduria | - |
Rattus norvegicus | - |
- |
- |
Purification (Comment) | Organism |
---|---|
partially purified from liver, further purification not possible due to the instability of the enzyme | Rattus norvegicus |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | low level | Rattus norvegicus | - |
heart | low level | Rattus norvegicus | - |
kidney | - |
Rattus norvegicus | - |
liver | - |
Rattus norvegicus | - |
additional information | traces of activity in muscle and spleen | Rattus norvegicus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
(S)-2-hydroxyglutarate + acceptor | - |
Homo sapiens | 2-oxoglutarate + reduced acceptor | - |
? | |
(S)-2-hydroxyglutarate + acceptor | enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria | Homo sapiens | 2-oxoglutarate + reduced acceptor | - |
? | |
(S)-2-hydroxyglutarate + FAD | - |
Rattus norvegicus | 2-oxoglutarate + FADH2 | - |
? | |
(S)-2-hydroxyglutarate + FAD | enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria | Rattus norvegicus | 2-oxoglutarate + FADH2 | - |
? | |
(S)-2-hydroxyglutarate + iodonitrotetrazolium | - |
Rattus norvegicus | 2-oxoglutarate + ? | - |
? | |
additional information | no activity with D-2-hydroxyglutarate, L-malate, and L-lactate | Rattus norvegicus | ? | - |
? |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
FAD | - |
Rattus norvegicus | |
additional information | no activation with FMN | Rattus norvegicus |