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Literature summary extracted from
- Recombinant heteromeric phenylalanine monooxygenase and the oxygenation of carbon and sulfur substrates (2011), J. Pharm. Pharmacol., 63, 558-564.
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 1.14.16.1 | expressed in Escherichia coli as a His-tagged fusion protein | Homo sapiens |
Protein Variants
| EC Number | Protein Variants | Comment | Organism |
|---|---|---|---|
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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 |
| 1.14.16.1 | 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]
| EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.14.16.1 | 0.1 | - |
L-Phe | allelic combination wild-type/mutant I65T, Vmax: 1 micromol/min/mg, pH 6.8, 37°C | Homo sapiens |  |
| 1.14.16.1 | 0.1 | - |
L-Phe | allelic combination wild-type/mutant R68S, Vmax: 1 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 0.1 | - |
L-Phe | allelic combination wild-type/wild-type, Vmax: 3 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 0.2 | - |
L-Phe | allelic combination wild-type/mutant Y414C, Vmax: 1 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 0.9 | - |
L-Phe | allelic combination wild-type/mutant V388M, Vmax: 0.9 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 1 | - |
L-Phe | allelic combination wild-type/mutant R158Q, Vmax: 0.9 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 1 | - |
L-Phe | allelic combination wild-type/mutant R261Q, Vmax: 0.9 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 2 | - |
L-Phe | allelic combination wild-type/mutant I174T, Vmax: 0.7 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 2 | 3 | S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R261Q, Vmax: 15 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 3 | - |
L-Phe | allelic combination wild-type/mutant R408W, Vmax: 0.7 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 8 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/wild-type, Vmax: 114 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 15 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant I65T, Vmax: 18 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 18 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R68S, Vmax: 17 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 19 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant I174T, Vmax: 2 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 20 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R158Q, Vmax: 3 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 21 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant R408W, Vmax: 2 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 24 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant Y414C, Vmax: 22 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
| 1.14.16.1 | 25 | - |
S-carboxymethyl-L-cysteine | allelic combination wild-type/mutant V388M, Vmax: 12 micromol/min/mg, pH 6.8, 37°C | Homo sapiens | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 1.14.16.1 | Homo sapiens | - |
- |
- |
Purification (Commentary)
| EC Number | Purification (Comment) | Organism |
|---|---|---|
| 1.14.16.1 | using Ni-NTA chromatography | Homo sapiens |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.14.16.1 | L-Phe + tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + dihydrobiopterin + H2O | - |
? | |
| 1.14.16.1 | S-carboxymethyl-L-cysteine + tetrahydrobiopterin + O2 | - |
Homo sapiens | S-carboxymethyl-L-cysteine S-oxide + dihydrobiopterin + H2O | - |
? | |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 1.14.16.1 | HPAH | - |
Homo sapiens |
| 1.14.16.1 | phenylalanine hydroxylase | - |
Homo sapiens |
Temperature Optimum [°C]
| EC Number | Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|---|
| 1.14.16.1 | 37 | - |
assay at | Homo sapiens |
pH Optimum
| EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|---|
| 1.14.16.1 | 6.8 | - |
assay at | Homo sapiens |
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Release 2023.1 (January 2023)
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