EC Number | Protein Variants | Comment | Organism |
---|---|---|---|
1.14.16.1 | I65T | the mutant shows increased specific activity using L-phenylalanine as substrate and decreased specific activity using S-carboxymethyl-L-cysteine compared to the wild type enzyme | Homo sapiens |
1.14.16.1 | R261Q | the mutant shows decreased specific activity using L-phenylalanine and S-carboxymethyl-L-cysteine as substrate compared to the wild type enzyme | Homo sapiens |
1.14.16.1 | R68S | the mutant shows decreased specific activity using L-phenylalanine and S-carboxymethyl-L-cysteine as substrate compared to the wild type enzyme | Homo sapiens |
1.14.16.1 | V388M | the mutant shows decreased specific activity using L-phenylalanine and S-carboxymethyl-L-cysteine as substrate compared to the wild type enzyme | Homo sapiens |
1.14.16.1 | Y414C | the mutant shows decreased specific activity using L-phenylalanine and S-carboxymethyl-L-cysteine as substrate compared to the wild type enzyme | Homo sapiens |
EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|---|
1.14.16.1 | 0.022 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme Y414C, using L-phenylalanine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.024 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme V388M, using L-phenylalanine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.026 | - |
5,6,7,8-tetrahydrobiopterin | wild type enzyme, using L-phenylalanine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.027 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme R261Q, using L-phenylalanine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.032 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme R68S, using L-phenylalanine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.04 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme I65T, using L-phenylalanine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.0728 | - |
5,6,7,8-tetrahydrobiopterin | wild type enzyme, using S-carboxymethyl-L-cysteine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.14 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme R68S, using S-carboxymethyl-L-cysteine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.143 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme Y414C, using S-carboxymethyl-L-cysteine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.146 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme V388M, using S-carboxymethyl-L-cysteine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.15 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme I65T, using S-carboxymethyl-L-cysteine as cosubstrate | Homo sapiens | |
1.14.16.1 | 0.155 | - |
5,6,7,8-tetrahydrobiopterin | mutant enzyme R261Q, using S-carboxymethyl-L-cysteine as cosubstrate | Homo sapiens | |
1.14.16.1 | 14.73 | - |
S-carboxymethyl-L-cysteine | wild type enzyme from hepatic cytosol | Rattus norvegicus | |
1.14.16.1 | 16.53 | - |
S-carboxymethyl-L-cysteine | wild type enzyme from hepatic cytosol | Homo sapiens | |
1.14.16.1 | 25.24 | - |
S-carboxymethyl-L-cysteine | wild type enzyme from Hep-G2 cell cytosol | Homo sapiens | |
1.14.16.1 | 43.25 | - |
S-methyl-L-cysteine | wild type enzyme from hepatic cytosol | Rattus norvegicus | |
1.14.16.1 | 44.63 | - |
S-methyl-L-cysteine | wild type enzyme from hepatic cytosol | Homo sapiens | |
1.14.16.1 | 51.6 | - |
S-methyl-L-cysteine | wild type enzyme from Hep-G2 cell cytosol | Homo sapiens | |
1.14.16.1 | 55.97 | - |
N-acetyl-S-carboxymethyl-L-cysteine | wild type enzyme from hepatic cytosol | Rattus norvegicus | |
1.14.16.1 | 57.15 | - |
N-acetyl-S-carboxymethyl-L-cysteine | wild type enzyme from hepatic cytosol | Homo sapiens | |
1.14.16.1 | 58.92 | - |
N-acetyl-S-methyl-L-cysteine | wild type enzyme from hepatic cytosol | Rattus norvegicus | |
1.14.16.1 | 60.54 | - |
N-acetyl-S-methyl-L-cysteine | wild type enzyme from hepatic cytosol | Homo sapiens | |
1.14.16.1 | 63.8 | - |
N-acetyl-S-carboxymethyl-L-cysteine | wild type enzyme from Hep-G2 cell cytosol | Homo sapiens | |
1.14.16.1 | 68.25 | - |
N-acetyl-S-methyl-L-cysteine | wild type enzyme from Hep-G2 cell cytosol | Homo sapiens |
EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
1.14.16.1 | cytosol | - |
Rattus norvegicus | 5829 | - |
1.14.16.1 | cytosol | - |
Homo sapiens | 5829 | - |
EC Number | Metals/Ions | Comment | Organism | Structure |
---|---|---|---|---|
1.14.16.1 | Fe2+ | contains one Fe2+ per monomer | Rattus norvegicus | |
1.14.16.1 | Fe2+ | contains one Fe2+ per monomer | Homo sapiens |
EC Number | Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|---|
1.14.16.1 | 52000 | - |
calculated from sequence of cDNA | Rattus norvegicus |
1.14.16.1 | 52000 | - |
calculated from sequence of cDNA | Homo sapiens |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
1.14.16.1 | Homo sapiens | P00439 | - |
- |
1.14.16.1 | Rattus norvegicus | - |
- |
- |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
1.14.16.1 | Hep-G2 cell | - |
Homo sapiens | - |
1.14.16.1 | liver | - |
Rattus norvegicus | - |
1.14.16.1 | liver | - |
Homo sapiens | - |
EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|---|
1.14.16.1 | 0.0009 | - |
mutant enzyme V388M, using S-carboxymethyl-L-cysteine as substrate | Homo sapiens |
1.14.16.1 | 0.0014 | - |
mutant enzyme I65T, using S-carboxymethyl-L-cysteine as substrate | Homo sapiens |
1.14.16.1 | 0.0014 | - |
mutant enzyme R68S, using S-carboxymethyl-L-cysteine as substrate | Homo sapiens |
1.14.16.1 | 0.0015 | - |
mutant enzyme Y414C, using S-carboxymethyl-L-cysteine as substrate | Homo sapiens |
1.14.16.1 | 0.0016 | - |
mutant enzyme R261Q, using S-carboxymethyl-L-cysteine as substrate | Homo sapiens |
1.14.16.1 | 0.073 | - |
wild type enzyme, using S-carboxymethyl-L-cysteine as substrate | Homo sapiens |
1.14.16.1 | 0.505 | - |
mutant enzyme V388M, using L-phenylalanine as substrate | Homo sapiens |
1.14.16.1 | 1.2 | - |
mutant enzyme Y414C, using L-phenylalanine as substrate | Homo sapiens |
1.14.16.1 | 1.49 | - |
mutant enzyme R261Q, using L-phenylalanine as substrate | Homo sapiens |
1.14.16.1 | 1.725 | - |
mutant enzyme R68S, using L-phenylalanine as substrate | Homo sapiens |
1.14.16.1 | 1.9 | - |
wild type enzyme, using L-phenylalanine as substrate | Homo sapiens |
1.14.16.1 | 2.25 | - |
mutant enzyme I65T, using L-phenylalanine as substrate | Homo sapiens |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.14.16.1 | L-phenylalanine + 5,6,7,8-tetrahydrobiopterin + O2 | - |
Rattus norvegicus | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
1.14.16.1 | L-phenylalanine + 5,6,7,8-tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
1.14.16.1 | additional information | thiodiglycolic acid is not a substrate for PAH | Rattus norvegicus | ? | - |
? | |
1.14.16.1 | additional information | thiodiglycolic acid is not a substrate for PAH | Homo sapiens | ? | - |
? | |
1.14.16.1 | N-acetyl-S-carboxymethyl-L-cysteine + O2 | - |
Rattus norvegicus | ? | - |
? | |
1.14.16.1 | N-acetyl-S-carboxymethyl-L-cysteine + O2 | - |
Homo sapiens | ? | - |
? | |
1.14.16.1 | N-acetyl-S-methyl-L-cysteine + O2 | - |
Rattus norvegicus | ? | - |
? | |
1.14.16.1 | N-acetyl-S-methyl-L-cysteine + O2 | - |
Homo sapiens | ? | - |
? | |
1.14.16.1 | S-carboxymethyl-L-cysteine + tetrahydrobiopterin + O2 | - |
Rattus norvegicus | S-carboxymethyl-L-cysteine S-oxide + dihydrobiopterin + H2O | - |
? | |
1.14.16.1 | S-carboxymethyl-L-cysteine + tetrahydrobiopterin + O2 | - |
Homo sapiens | S-carboxymethyl-L-cysteine S-oxide + dihydrobiopterin + H2O | - |
? | |
1.14.16.1 | S-methyl-L-cysteine + O2 | - |
Rattus norvegicus | ? | - |
? | |
1.14.16.1 | S-methyl-L-cysteine + O2 | - |
Homo sapiens | ? | - |
? |
EC Number | Subunits | Comment | Organism |
---|---|---|---|
1.14.16.1 | homotetramer | - |
Homo sapiens |
1.14.16.1 | homotetramer | 4 * 52000, calculated from sequence of cDNA | Rattus norvegicus |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
1.14.16.1 | PAH | - |
Rattus norvegicus |
1.14.16.1 | PAH | - |
Homo sapiens |
1.14.16.1 | phenylalanine hydroxylase | - |
Rattus norvegicus |
1.14.16.1 | phenylalanine hydroxylase | - |
Homo sapiens |
EC Number | Cofactor | Comment | Organism | Structure |
---|---|---|---|---|
1.14.16.1 | 5,6,7,8-tetrahydro-L-biopterin | - |
Rattus norvegicus | |
1.14.16.1 | 5,6,7,8-tetrahydro-L-biopterin | - |
Homo sapiens |
EC Number | General Information | Comment | Organism |
---|---|---|---|
1.14.16.1 | physiological function | key enzyme in the sulfoxidation of S-carboxymethyl-L-cysteine S-oxide and its thioester metabolites S-methyl-L-cysteine, N-acetyl-S-carboxymethyl-L-cysteine, and N-acetyl-S-methyl-L-cysteine | Rattus norvegicus |
1.14.16.1 | physiological function | key enzyme in the sulfoxidation of S-carboxymethyl-L-cysteine S-oxide and its thioester metabolites S-methyl-L-cysteine, N-acetyl-S-carboxymethyl-L-cysteine, and N-acetyl-S-methyl-L-cysteine | Homo sapiens |