Activating Compound | Comment | Organism | Structure |
---|---|---|---|
L-phenylalanine | - |
Homo sapiens |
Cloned (Comment) | Organism |
---|---|
wild type and mutant enzyme S231F are expressed in Escherichia coli BL21(DE3) and human hepatoma cells, in the cells supplemented with sepiapterin the amount of expressed S231F PAH increases up to 25% | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
S231F | the missense phenylalanine hydroxylase gene mutation causes complete loss of enzymatic activity in vitro (residual enzyme activity in vitro is about 1%) as it drastically reduces stability and activity of the PAH enzyme, the mutant enzyme is not activated by pre-incubation with L-phenylalanine substrate | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
94000 | - |
approximately 94000 Da, SDS-PAGE | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P00439 | - |
- |
Purification (Comment) | Organism |
---|---|
amylose column chromatography | Homo sapiens |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
0.0049 | - |
L-phenylalanine-activated mutant enzyme S231F, at 25°C | Homo sapiens |
0.0052 | - |
non-L-phenylalanine-activated mutant enzyme S231F, at 25°C | Homo sapiens |
0.4169 | - |
non-L-phenylalanine-activated wild type enzyme, at 25°C | Homo sapiens |
0.7418 | - |
L-phenylalanine-activated wild type enzyme, at 25°C | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-phenylalanine + 5,6,7,8-tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? |
Synonyms | Comment | Organism |
---|---|---|
PAH | - |
Homo sapiens |
phenylalanine hydroxylase | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
5,6,7,8-tetrahydro-L-biopterin | - |
Homo sapiens |