Cloned (Comment) | Organism |
---|---|
enzyme expression by in vitro transcription-translation using a rapid translation system with enhanced Escherichia coli lysate and radio-labeled L-Met, tetrahydrobioterin increases the enzyme protein yield | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
L205P | the mutant is associated with recessively inherited L-DOPA-responsive infantile parkinsonism, the mutation reduces the activity and stability of the protein in cells and in vitro expression systems, being considered a misfolding mutation | Homo sapiens |
General Stability | Organism |
---|---|
binding of inhibitor dopamine and of cofactor tetrahydrobioterin stabilizes the enzyme | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin | i.e. BH4, cofactor, complex regulation by the cofactor including both enzyme inactivation and conformational stabilization, competitive inhibition, synergistically with DTT | Homo sapiens | |
dopamine | feedback inhibitor and catecholamine product, binding inhibits and stabilizes the enzyme, reversible and competitive inhibition with respect to tetrahydrobiopterin | Homo sapiens | |
DTT | inactivates the enzyme, synergistically with tetrahydrobiopterin | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
additional information | - |
additional information | both the cofactor (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin and the feedback inhibitor dopamine increase the kinetic stability of human isozyme 1 in vitro | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
58000 | - |
x * 58000, recombinant enzyme, SDS-PAGE | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-tyrosine + tetrahydrobiopterin + O2 | Homo sapiens | rate-limiting enzyme in the synthesis of catecholamine neurotransmitters, complex regulation by the cofactor including both enzyme inactivation and conformational stabilization, enzyme defects are associated with L-DOPA responsive and non-responsive dystonia and infantile parkinsonism, overview | 3,4-dihydroxy-L-phenylalanine + 4a-hydroxytetrahydrobiopterin + H2O | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
isozyme TH 1 | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
neuron | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-tyrosine + tetrahydrobiopterin + O2 | - |
Homo sapiens | 3,4-dihydroxy-L-phenylalanine + 4a-hydroxytetrahydrobiopterin + H2O | - |
? | |
L-tyrosine + tetrahydrobiopterin + O2 | rate-limiting enzyme in the synthesis of catecholamine neurotransmitters, complex regulation by the cofactor including both enzyme inactivation and conformational stabilization, enzyme defects are associated with L-DOPA responsive and non-responsive dystonia and infantile parkinsonism, overview | Homo sapiens | 3,4-dihydroxy-L-phenylalanine + 4a-hydroxytetrahydrobiopterin + H2O | - |
? |
Subunits | Comment | Organism |
---|---|---|
? | x * 58000, recombinant enzyme, SDS-PAGE | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
tyrosine hydroxylase | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
25 | - |
assay at, recombinant enzyme | Homo sapiens |
30 | - |
assay at, crude brain enzyme extract | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7 | - |
assay at | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin | i.e. BH4, complex regulation by the cofactor including both enzyme inactivation and conformational stabilization, competitive inhibition | Homo sapiens |
Ki Value [mM] | Ki Value maximum [mM] | Inhibitor | Comment | Organism | Structure |
---|---|---|---|---|---|
0.07 | - |
(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin | pH 7.0, 25°C, versus (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin | Homo sapiens |