Cloned (Comment) | Organism |
---|---|
several pyridoxamine 5'-phosphate oxidase pathogenic variants responsible for pyridoxamine 5'-phosphate oxidase deficiency (G118R, R141C, R225H, R116Q/R225H, and X262Q) are recombinantly expressed in Escherichia coli | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
G118R | pathogenic variant that is responsible for pyridoxamine 5'-phosphate oxidase deficiency. 51% of the activity as compared to wild-type enzyme. Tm-value of the mutant enzyme (in absence and presence of FMN) is about 10°C lower than the Tm-value of the wilde-type enzyme | Homo sapiens |
R116Q | 83% of the activity as compared to wild-type enzyme | Homo sapiens |
R116Q | the Tm-value of the mutant enzyme in absence of FMN is 7.2°C lower than the Tm-value of the wilde-type enzyme in absence of FMN. The Tm-value of the mutant enzyme in absence of FMN is 8.2°C lower than the Tm-value of the wilde-type enzyme in presence of FMN | Homo sapiens |
R116Q/R225H | pathogenic variant that is responsible for pyridoxamine 5'-phosphate oxidase deficiency. The Tm-value of the mutant enzyme in absence of FMN is 5.3°C lower than the Tm-value of the wilde-type enzyme in absence of FMN. The Tm-value of the mutant enzyme in absence of FMN is 4.7°C lower than the Tm-value of the wilde-type enzyme in presence of FMN | Homo sapiens |
R141C | pathogenic variant that is responsible for pyridoxamine 5'-phosphate oxidase deficiency. The Tm-value of the mutant enzyme in absence of FMN is 6.2°C lower than the Tm-value of the wilde-type enzyme in absence of FMN. The Tm-value of the mutant enzyme in absence of FMN is 6.0°C lower than the Tm-value of the wilde-type enzyme in presence of FMN | Homo sapiens |
R225H | pathogenic variant that is responsible for pyridoxamine 5'-phosphate oxidase deficiency. The Tm-value of the mutant enzyme in absence of FMN is 5.1°C higher than the Tm-value of the wilde-type enzyme in absence of FMN. The Tm-value of the mutant enzyme in absence of FMN is 3.8°C lower than the Tm-value of the wilde-type enzyme in presence of FMN | Homo sapiens |
R229W | 15-30% of the activity as compared to wild-type enzyme | Homo sapiens |
X262Q | pathogenic variant that is responsible for pyridoxamine 5'-phosphate oxidase deficiency. The Tm-value of the mutant enzyme in absence of FMN is 2.7°C lower than the Tm-value of the wilde-type enzyme in absence of FMN. The Tm-value of the mutant enzyme in absence of FMN is 4.7°C lower than the Tm-value of the wilde-type enzyme in presence of FMN | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
pyridoxal 5'-phosphate | - |
Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.0026 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, wild-type enzyme, TRIS buffer | Homo sapiens | |
0.0028 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, wild-type enzyme, HEPES buffer | Homo sapiens | |
0.0031 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R116Q | Homo sapiens | |
0.0051 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R116Q/R225H | Homo sapiens | |
0.0057 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme G118R | Homo sapiens | |
0.0061 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R141C | Homo sapiens | |
0.016 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R225H | Homo sapiens | |
0.436 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R95C | Homo sapiens | |
0.461 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R229W | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q9NVS9 | - |
- |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
pyridoxine 5'-phosphate + O2 | - |
Homo sapiens | pyridoxal 5'-phosphate + H2O2 | - |
? |
Subunits | Comment | Organism |
---|---|---|
homodimer | - |
Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
PNPO | - |
Homo sapiens |
pyridoxine 5'-phosphate oxidase | - |
Homo sapiens |
Temperature Stability Minimum [°C] | Temperature Stability Maximum [°C] | Comment | Organism |
---|---|---|---|
44.3 | - |
Tm-value in presence of FMN, mutant enzyme G118R | Homo sapiens |
48.2 | - |
Tm-value in presence of FMN, mutant enzyme R116Q | Homo sapiens |
48.7 | - |
Tm-value in presence of FMN, mutant enzyme G118R | Homo sapiens |
49.2 | - |
Tm-value in presence of FMN, mutant enzyme R141C | Homo sapiens |
50.1 | - |
Tm-value in presence of FMN, mutant enzyme R116Q | Homo sapiens |
50.1 | - |
Tm-value in presence of FMN, mutant enzyme R116Q/R225H | Homo sapiens |
52.3 | - |
Tm-value in presence of FMN, mutant enzyme R141C | Homo sapiens |
52.7 | - |
Tm-value in presence of FMN, mutant enzyme X262Q | Homo sapiens |
53.7 | - |
Tm-value in presence of FMN, mutant enzyme R116Q/R225H | Homo sapiens |
55.4 | - |
Tm-value in presence of FMN, wild-type enzyme | Homo sapiens |
55.9 | - |
Tm-value in presence of FMN, mutant enzyme X262Q | Homo sapiens |
58.3 | - |
Tm-value in presence of FMN, wild-type enzyme | Homo sapiens |
60.5 | - |
Tm-value in presence of FMN, mutant enzyme R225H | Homo sapiens |
62.1 | - |
Tm-value in presence of FMN, mutant enzyme R225H | Homo sapiens |
Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.002 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R225H | Homo sapiens | |
0.012 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R116Q/R225H | Homo sapiens | |
0.019 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R141C | Homo sapiens | |
0.022 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme G118R | Homo sapiens | |
0.037 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R116Q | Homo sapiens | |
0.037 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R95C | Homo sapiens | |
0.04 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, mutant enzyme R229W | Homo sapiens | |
0.057 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, wild-type enzyme, HEPES buffer | Homo sapiens | |
0.062 | - |
pyridoxine 5'-phosphate | pH 7.6, 37°C, wild-type enzyme, TRIS buffer | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
pyridoxal 5'-phosphate | the enzyme has an allosteric pyridoxal 5'-phosphate binding site that plays a crucial role in the enzyme regulation and therefore in the regulation of vitamin B6 metabolism in humans | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | the pathogenic forms of pyridoxine 5'-phosphate oxidase is associated with neonatal epileptic encephalopathy | Homo sapiens |