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Literature summary for 1.14.16.1 extracted from
- A new model for allosteric regulation of phenylalanine hydroxylase: implications for disease and therapeutics (2013), Arch. Biochem. Biophys., 530, 73-82.
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 50000 | - |
4 * 50000, SDS-PAGE | Homo sapiens |
| 50000 | - |
4 * 50000, SDS-PAGE | Rattus norvegicus |
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 | - |
? |  |
| L-phenylalanine + tetrahydrobiopterin + O2 | Rattus norvegicus | - |
L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
| Rattus norvegicus | P04176 | - |
- |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| HiTrap Q column chromatography | Homo sapiens |
| phenyl Sepharose column chromatography | Rattus norvegicus |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| L-phenylalanine + tetrahydrobiopterin + O2 | - |
Homo sapiens | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
| L-phenylalanine + tetrahydrobiopterin + O2 | - |
Rattus norvegicus | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| tetramer | 4 * 50000, SDS-PAGE | Homo sapiens |
| tetramer | 4 * 50000, SDS-PAGE | Rattus norvegicus |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| PAH | - |
Homo sapiens |
| PAH | - |
Rattus norvegicus |
| phenylalanine hydroxylase | - |
Homo sapiens |
| phenylalanine hydroxylase | - |
Rattus norvegicus |
General Information
| General Information | Comment | Organism |
|---|---|---|
| malfunction | phenylalanine hydroxylase dysfunction causes phenylketonuria | Homo sapiens |
| malfunction | phenylalanine hydroxylase dysfunction causes phenylketonuria | Rattus norvegicus |
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Release 2023.1 (January 2023)
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