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Literature summary for 1.4.3.5 extracted from

  • Barile, A.; Nogues, I.; di Salvo, M.L.; Bunik, V.; Contestabile, R.; Tramonti, A.
    Molecular characterization of pyridoxine 5-phosphate oxidase and its pathogenic forms associated with neonatal epileptic encephalopathy (2020), Sci. Rep., 10, 13621 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 1.4.3.5

Cloned(Commentary)

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

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

Inhibitors Comment Organism Structure
pyridoxal 5'-phosphate
-
 Homo sapiens

KM Value [mM]

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

Organism UniProt Comment Textmining
Homo sapiens Q9NVS9
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
pyridoxine 5'-phosphate + O2
-
 Homo sapiens pyridoxal 5'-phosphate + H2O2
-
 ?

Subunits

Subunits Comment Organism
homodimer
-
 Homo sapiens

Synonyms

Synonyms Comment Organism
PNPO
-
 Homo sapiens
pyridoxine 5'-phosphate oxidase
-
 Homo sapiens

Temperature Stability [°C]

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 [1/s]

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

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

General Information Comment Organism
malfunction the pathogenic forms of pyridoxine 5'-phosphate oxidase is associated with neonatal epileptic encephalopathy Homo sapiens