PAH Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; EC 1.14.16.1; Oxidoreductase
Chromosomal Location of human Ortholog: 12q23.2
Cellular Component:  cytosol
Molecular Function:  iron ion binding; phenylalanine 4-monooxygenase activity
Biological Process:  amino acid biosynthetic process; catecholamine biosynthetic process; L-phenylalanine catabolic process; neurotransmitter biosynthetic process; tyrosine biosynthetic process; tyrosine biosynthetic process, by oxidation of phenylalanine
Disease: Phenylketonuria
Reference #:  P00439 (UniProtKB)
Alt. Names/Synonyms: PAH; PH; PH4H; Phe-4-monooxygenase; phenylalanine 4-monooxygenase; phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; PKU1
Gene Symbols: PAH
Molecular weight: 51,862 Da
Basal Isoelectric point: 6.15  Predict pI for various phosphorylation states
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PAH

Protein Structure Not Found.


Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein