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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