iPTMnet Report for P00439 (PAH)


Protein Information

Cytoscape View


UniProt AC / UniProt ID P00439 / PH4H_HUMAN
Protein Name Phenylalanine-4-hydroxylase
Gene Name Name: PAH
Organism Homo sapiens (Human)

PRO ID PR:P00439
DAG View
PRO Name phenylalanine-4-hydroxylase (human)
Definition A phenylalanine-4-hydroxylase that is encoded in the genome of human.
Short Label hPAH
Category organism-gene

Interactive Sequence View

Select/align proteoforms across species


P00439 (PAH) as Substrate



Site PTM Type PTM Enzyme Score Source PMID
S2 Phosphorylation PhosphoSitePlus
T3 Phosphorylation PhosphoSitePlus
S16 Phosphorylation IEDB   phospho.ELM   PhosphoSitePlus   RLIMS-P   UniProt 20937381, 8573072, 2451499, ...12185072, 12554741, 15096628, 24275569, 22005392, 22371403
S16 Phosphorylation P17612 (PRKACA) PhosphoSitePlus 20937381, 12185072
S87 Phosphorylation PhosphoSitePlus
T92 Phosphorylation PhosphoSitePlus
T117 Phosphorylation PhosphoSitePlus
C265 Methylation IEDB 31844290
S436 Phosphorylation PhosphoSitePlus
C445 Methylation IEDB 31844290


PTM sites affected in variants

Site Variant Source PMID Disease [Sample source]
S16 F16 Biomuta DOID:3571 / liver cancer [ cosmic, icgc ]
S16 Y16 Biomuta DOID:4159 / skin cancer [ cosmic, icgc ]
C265 F265 Biomuta DOID:1324 / lung cancer [ cosmic ]
S436 Y436 Biomuta DOID:3571 / liver cancer [ cosmic ]
S436 F436 Biomuta DOID:4159 / skin cancer [ cosmic ]
C445 F445 Biomuta DOID:9256 / colorectal cancer [ cosmic, icgc ]