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Literature summary for 1.14.16.1 extracted from
- Probing the role of crystallographically defined/predicted hinge-bending regions in the substrate-induced global conformational transition and catalytic activation of human phenylalanine hydroxylase by single-site mutagenesis (2004), J. Biol. Chem., 279, 26571-26580.
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| G33A | increased basal activity, reduced activation by preincubation with substrate | Homo sapiens |
| G33V | increased basal activity, reduced activation by preincubation with substrate | Homo sapiens |
| K113P | increased basal activity, reduced activation by preincubation with substrate, increase in positive cooperativity | Homo sapiens |
| N223D | low basal activity, little activation by preincubation with substrate, increase in positive cooperativity | Homo sapiens |
| N32D | low basal activity, close to normal activation by preincubation with substrate | Homo sapiens |
| N426D | low basal activity, close to normal activation by preincubation with substrate | Homo sapiens |
| T427P | low basal activity, little activation by preincubation with substrate, no kinetic cooperativity | Homo sapiens |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | P00439 | - |
- |
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| 1.98 | - |
substrate L-phenylalanine, mutant N223D | Homo sapiens |
| 2.34 | - |
substrate tetrahydrobiopterin, mutant N223D | Homo sapiens |
| 2.84 | - |
substrate tetrahydrobiopterin, mutant T427P | Homo sapiens |
| 3.17 | - |
substrate L-phenylalanine, mutant T427P | Homo sapiens |
| 3.48 | - |
substrate L-phenylalanine, wild-type | Homo sapiens |
| 3.67 | - |
substrate L-phenylalanine, mutant N426D | Homo sapiens |
| 3.74 | - |
substrate L-phenylalanine, mutant N32D | Homo sapiens |
| 4.37 | - |
substrate L-phenylalanine, mutant G33A | Homo sapiens |
| 4.39 | - |
substrate tetrahydrobiopterin, mutant N426D | Homo sapiens |
| 4.73 | - |
substrate tetrahydrobiopterin, mutant G33A | Homo sapiens |
| 4.73 | - |
substrate tetrahydrobiopterin, mutant N32D | Homo sapiens |
| 5.16 | - |
substrate L-phenylalanine, mutant G33V | Homo sapiens |
| 5.32 | - |
substrate tetrahydrobiopterin, wild-type | Homo sapiens |
| 6.19 | - |
substrate L-phenylalanine, mutant K113P | Homo sapiens |
| 6.41 | - |
substrate tetrahydrobiopterin, mutant G33V | Homo sapiens |
| 8.56 | - |
substrate tetrahydrobiopterin, mutant K113P | Homo sapiens |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| L-phenylalanine + tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + dihydrobiopterin + H2O | - |
? |  |
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