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Tclin
PAH
Phenylalanine-4-hydroxylase

Protein Summary
Description
Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine. This gene encodes a member of the biopterin-dependent aromatic amino acid hydroxylase protein family. The encoded phenylalanine hydroxylase enzyme hydroxylates phenylalanine to tyrosine and is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Aug 2017]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000553106
  • ENSP00000448059
  • ENSG00000171759

Symbol
  • PH
  • PKU
  • PKU1
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
protein domain
0.85
molecular function
0.63
biological term
0.62
drug
0.6
gene perturbation
0.6


IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 363.8   (req: < 5)
Gene RIFs: 135   (req: <= 3)
Antibodies: 260   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 363.8   (req: >= 5)
Gene RIFs: 135   (req: > 3)
Antibodies: 260   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 7
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 1
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 1
Approved Drugs (1)
1 – 1 of 1
sapropterin
chemical structure image
Active Ligands (1)
1 – 1 of 1
CHEMBL187878
chemical structure image
Protein Data Bank (17)
1 – 5 of 17
PDB Structure Id
Ligand
Method
Resolution (Å)
M.W. (kDa)
Pub Year
Title
PDB Structure Id
M.W.
Resolution
Pub Year
Pathways (27)
Disease (R-HSA-1643685)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 5 of 7
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Disease
Reactome
Diseases of metabolism
Reactome
Metabolism
Reactome
Metabolism of amino acids and derivatives
Reactome
Phenylalanine and tyrosine metabolism
Name
Explore in Pharos
Explore in Source
Disease
Diseases of metabolism
Metabolism
Metabolism of amino acids and derivatives
Phenylalanine and tyrosine metabolism
Gene Ontology Terms (8)
Items per page:
10
1 – 2 of 2
GO Term
Evidence
Assigned by
Inferred from Direct Assay (IDA)
CACAO
Inferred from Electronic Annotation (IEA)
InterPro
Protein-Protein Interactions (128)
1 – 10 of 128
QDPR
Tchem
Family: Enzyme
Novelty: 0.00920882
Score: 0.994
Data Source: STRINGDB
PCBD1
Tbio
Family: Enzyme
Novelty: 0.01326826
Score: 0.984
Data Source: STRINGDB
SPR
Tchem
Family: Enzyme
Novelty: 0.02634716
Score: 0.98
Data Source: STRINGDB
TAT
Tbio
Family: Enzyme
Novelty: 0.00082991
Score: 0.978
Data Source: STRINGDB
DDC
Tclin
Family: Enzyme
Novelty: 0.0016806
Score: 0.971
Data Source: STRINGDB
HGD
Tbio
Family: Enzyme
Novelty: 0.00213809
Score: 0.945
Data Source: STRINGDB
DHFR
Tclin
Family: Enzyme
Novelty: 0.0010094
Score: 0.945
Data Source: STRINGDB
GOT1
Tbio
Family: Enzyme
Novelty: 0.00607491
Score: 0.924
Data Source: STRINGDB
PCBD2
Tdark
Family: Enzyme
Novelty: 0.19498607
Score: 0.923
Data Source: STRINGDB
AKR1B1
Tclin
Family: Enzyme
Novelty: 0.00050572
Score: 0.92
Data Source: STRINGDB
Publication Statistics
PubMed Score  363.80

PubMed score by year
PubTator Score  1467.85

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer
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