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Literature summary for 1.14.16.1 extracted from
- PKU mutation p.G46S prevents the stereospecific binding of L-phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain (2017), FEBS open bio, 7, 195-203 .
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| the catalytic domain is expressed in Escherichia coli BL21(DE3) cells | Homo sapiens |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| G46S | the mutation causes phenylketonuria | Homo sapiens |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 156000 | - |
gel filtration | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| L-phenylalanine + tetrahydrobiopterin + O2 | Homo sapiens | - |
L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | P00439 | - |
- |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| L-phenylalanine + tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| homodimer | 2 * 65000, catalytic domain fused to maltose-binding protein, SDS-PAGE | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| PAH | - |
Homo sapiens |
| phenylalanine hydroxylase | - |
Homo sapiens |
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Release 2023.1 (January 2023)
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