Cloned (Comment) | Organism |
---|---|
expressed in Escherichia coli as a His-tagged fusion protein | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
I174T | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine or L-Phe) compared to wild-type | Homo sapiens |
I65T | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine) no difference in Km (L-Phe) compared to wild-type | Homo sapiens |
R158Q | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine or L-Phe) compared to wild-type | Homo sapiens |
R261Q | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine) no difference in Km (L-Phe) compared to wild-type | Homo sapiens |
R408W | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine or L-Phe) compared to wild-type | Homo sapiens |
R68S | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine) no difference in Km (L-Phe) compared to wild-type | Homo sapiens |
V388M | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine or L-Phe) compared to wild-type | Homo sapiens |
Y414C | heteromeric hPAH (wild-type + mutant) shows: significantly decreased Vmax values compared to wild-type, significantly increased Km values (substrate: S-carboxymethyl-L-cysteine or L-Phe) compared to wild-type | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.1 | - |
L-Phe | allelic combination wild-type/mutant I65T, Vmax: 1 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
0.1 | - |
L-Phe | allelic combination wild-type/mutant R68S, Vmax: 1 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
0.1 | - |
L-Phe | allelic combination wild-type/wild-type, Vmax: 3 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
0.2 | - |
L-Phe | allelic combination wild-type/mutant Y414C, Vmax: 1 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
0.9 | - |
L-Phe | allelic combination wild-type/mutant V388M, Vmax: 0.9 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
1 | - |
L-Phe | allelic combination wild-type/mutant R158Q, Vmax: 0.9 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
1 | - |
L-Phe | allelic combination wild-type/mutant R261Q, Vmax: 0.9 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
2 | - |
L-Phe | allelic combination wild-type/mutant I174T, Vmax: 0.7 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
2 | 3 | S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R261Q, Vmax: 15 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
3 | - |
L-Phe | allelic combination wild-type/mutant R408W, Vmax: 0.7 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
8 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/wild-type, Vmax: 114 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
15 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant I65T, Vmax: 18 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
18 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R68S, Vmax: 17 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
19 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant I174T, Vmax: 2 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
20 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R158Q, Vmax: 3 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
21 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R408W, Vmax: 2 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
24 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant Y414C, Vmax: 22 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
25 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant V388M, Vmax: 12 micromol/min/mg, pH 6.8, 37°C | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
using Ni-NTA chromatography | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-Phe + tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + dihydrobiopterin + H2O | - |
? | |
S-carboxymethyl-L-cysteine + tetrahydrobiopterin + O2 | - |
Homo sapiens | S-carboxymethyl-L-cysteine S-oxide + dihydrobiopterin + H2O | - |
? |
Synonyms | Comment | Organism |
---|---|---|
HPAH | - |
Homo sapiens |
phenylalanine hydroxylase | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
6.8 | - |
assay at | Homo sapiens |