EC Number   |
Natural Substrates |
|---|
    1.14.16.1 | L-phenylalanine + (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + O2 |
- |
| 1.14.16.1 | L-phenylalanine + (6R)-tetrahydrobiopterin + O2 |
in mammals rate-limiting step in complete catabolism of phenylalanine to CO2 and water |
| 1.14.16.1 | L-phenylalanine + 5,6,7,8-tetrahydrobiopterin + O2 |
- |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
- |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
modelling of cellular regulation |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
enzyme mutations can lead to phenylketonuria and BH4-responsive hyperphenylalaninemia in case of enzyme deficiency |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
enzyme mutations can lead to phenylketonuria, overview |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
impact of the enzyme structure on its regulation, overview |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
mechanism and regulation of the enzyme, which is involved in synthesis of a melamin compound required for cuticle synthesis, overview |
| 1.14.16.1 | L-phenylalanine + tetrahydrobiopterin + O2 |
mutations in the pah gene, e.g. manifestating in the regulatory domain, can lead to phenylketonuria, patients respond to treatment with tetrahydrobiopterin, overview |
