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Literature summary for 1.14.16.1 extracted from
- Effects of tetrahydrobiopterin and phenylalanine on in vivo human phenylalanine hydroxylase by phenylalanine breath test (2007), Mol. Genet. Metab., 92, 308-314.
Activating Compound
| Activating Compound | Comment | Organism | Structure |
|---|---|---|---|
| tetrahydrobiopterin | excessive dosages of BH4 inhibit PAH under normal phenylalanine conditions in vivo and activate PAH under conditions of high phenylalanine, overview | Homo sapiens |  |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| tetrahydrobiopterin | excessive dosages of BH4 inhibit PAH under normal phenylalanine conditions in vivo and activate PAH under conditions of high phenylalanine, overview | 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 | the native regulation system is designed to maintain phenylalanine levels constant in the human body | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
gene pah | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| additional information | - |
phenlyalanine level determination by the phenylalanine breath test, overview | Homo sapiens |
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 | the native regulation system is designed to maintain phenylalanine levels constant in the human body | Homo sapiens | L-tyrosine + 4a-hydroxytetrahydrobiopterin | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| PAH | - |
Homo sapiens |
| phenylalanine hydroxylase | - |
Homo sapiens |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| tetrahydrobiopterin | - |
Homo sapiens | |
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Release 2023.1 (January 2023)
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