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Literature summary extracted from
- Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias (2005), J. Inherit. Metab. Dis., 28, 921-930.
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.1.99.24 | 3-hydroxybutyrate + 2-oxoglutarate | Homo sapiens | - |
succinic semialdehyde + D-2-hydroxyglutarate | - |
r |  |
| 1.1.99.24 | additional information | Homo sapiens | D-2-hydroxyglutaric aciduria is not associated to the enzyme activity | ? | - |
? | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 1.1.99.24 | Homo sapiens | - |
- |
- |
Source Tissue
| EC Number | Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|---|
| 1.1.99.24 | fibroblast | of healthy subjects and patients with D-2-hydroxyglutaric aciduria | Homo sapiens | - |
| 1.1.99.24 | liver | - |
Homo sapiens | - |
Specific Activity [micromol/min/mg]
| EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|---|
| 1.1.99.24 | additional information | - |
assay method optimization, overview | Homo sapiens |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.1.99.24 | 3-hydroxybutyrate + 2-oxoglutarate | - |
Homo sapiens | succinic semialdehyde + D-2-hydroxyglutarate | - |
r | |
| 1.1.99.24 | 3-hydroxybutyrate + 2-oxoglutarate | radiolabeled substrate 3-hydroxybutyrate, stereospecific reaction | Homo sapiens | succinic semialdehyde + D-2-hydroxyglutarate | radiolabeled product D-2-hydroxyglutarate | r | |
| 1.1.99.24 | additional information | D-2-hydroxyglutaric aciduria is not associated to the enzyme activity | Homo sapiens | ? | - |
? | |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 1.1.99.24 | HOT | - |
Homo sapiens |
Temperature Optimum [°C]
| EC Number | Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|---|
| 1.1.99.24 | 37 | - |
assay at | Homo sapiens |
pH Optimum
| EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|---|
| 1.1.99.24 | 7.5 | - |
assay at | Homo sapiens |
Cofactor
| EC Number | Cofactor | Comment | Organism | Structure |
|---|---|---|---|---|
| 1.1.99.24 | additional information | no cofactor required | Homo sapiens |
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Release 2023.1 (January 2023)
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