ENTRY       EC 1.14.16.1
NAME        Phenylalanine 4-monooxygenase
            Phenylalaninase
            Phenylalanine 4-hydroxylase
CLASS       Oxidoreductases
            Acting on paired donors with incorporation of molecular oxygen
            With reduced pteridine as one donor, and incorporation of
            one atom of oxygen
SYSNAME     L-Phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase
            (4-hydroxylating)
REACTION    L-Phenylalanine + Tetrahydrobiopterin + O2 = L-Tyrosine +
            Dihydrobiopterin + H2O
SUBSTRATE   L-Phenylalanine
            Tetrahydrobiopterin
            O2
PRODUCT     L-Tyrosine
            Dihydrobiopterin
            H2O
COFACTOR    Iron
COMMENT     The mammalian enzyme is an iron protein.  Formerly EC 1.14.3.1
            and 1.99.1.2.
PATHWAY     PATH: MAP00400  Phenylalanine, tyrosine and tryptophan biosynthesis
DISEASE     MIM: 261600  Phenylalanine hydroxylase; Phenylketonuria (3);
                         [Hyperphenylalaninemia, mild] (3)
MOTIF       PS: PS00367  P-D-x(2)-H-[DE]-[LI]-[LIVMF]-G-H-[LIVMC]-P
GENES       HSA: PAH(Hs.1870)
DBLINKS     University of Geneva ENZYME DATA BANK: 1.14.16.1
            WIT (What Is There) Metabolic Reconstruction: 1.14.16.1
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