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Crystal structure of ternary complex of the catalytic domain of human phenylalanine hydroxylase ((FeII)) complexed with tetrahydrobiopterin and thienylalanine

Source organism: Homo sapiens
Biochemical function: oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced pteridine as one donor, and incorporation of one atom of oxygen
Biological process: aromatic amino acid metabolic process
Cellular component: not assigned

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EC 1.14.16.1: Phenylalanine 4-monooxygenase

Reaction catalysed:
L-phenylalanine + tetrahydrobiopterin + O(2) = L-tyrosine + 4a-hydroxytetrahydrobiopterin
Systematic name:
L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (4-hydroxylating)
Alternative Name(s):
  • PAH
  • Phenylalaninase
  • Phenylalanine 4-hydroxylase
  • Phenylalanine hydroxylase

GO terms

Sequence family

Pfam Protein family (Pfam)
PF00351
Domain description: Biopterin-dependent aromatic amino acid hydroxylase
Occurring in:
  1. Phenylalanine-4-hydroxylase
The deposited structure of PDB entry 1mmk contains 1 copy of Pfam domain PF00351 (Biopterin-dependent aromatic amino acid hydroxylase) in Phenylalanine-4-hydroxylase. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR019774
Domain description: Aromatic amino acid hydroxylase, C-terminal
Occurring in:
  1. Phenylalanine-4-hydroxylase
IPR018301
Domain description: Aromatic amino acid hydroxylase, iron/copper binding site
Occurring in:
  1. Phenylalanine-4-hydroxylase
IPR041912
Domain description: Eukaryotic phenylalanine-4-hydroxylase, catalytic domain
Occurring in:
  1. Phenylalanine-4-hydroxylase
IPR036329
Domain description: Aromatic amino acid monoxygenase, C-terminal domain superfamily
Occurring in:
  1. Phenylalanine-4-hydroxylase
IPR001273
Domain description: Aromatic amino acid hydroxylase
Occurring in:
  1. Phenylalanine-4-hydroxylase
IPR036951
Domain description: Aromatic amino acid hydroxylase superfamily
Occurring in:
  1. Phenylalanine-4-hydroxylase

Structure domain

CATH CATH domain
1.10.800.10
Class: Mainly Alpha
Architecture: Orthogonal Bundle
Topology: Phenylalanine Hydroxylase
Homology: Aromatic amino acid hydroxylase
Occurring in:
  1. Phenylalanine-4-hydroxylase
The deposited structure of PDB entry 1mmk contains 1 copy of CATH domain 1.10.800.10 (Phenylalanine Hydroxylase) in Phenylalanine-4-hydroxylase. Showing 1 copy in chain A.
SCOP SCOP annotation
56535
Class: Alpha and beta proteins (a+b)
Fold: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains
Superfamily: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains
Occurring in:
  1. Phenylalanine-4-hydroxylase
The deposited structure of PDB entry 1mmk contains 1 copy of SCOP domain 56535 (Aromatic aminoacid monoxygenases, catalytic and oligomerization domains) in Phenylalanine-4-hydroxylase. Showing 1 copy in chain A.

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