Protein Name phenylalanine hydroxylase
Organism Homo sapiens
Gene ID 5053
Gene Symbol


UniProt P00439 (PH4H_HUMAN), A0A024RBG4 (A0A024RBG4_HUMAN)
Relationships Total Number of functionally related compound(s) : 991
Total Number of Articles : 1442

phenylalanine hydroxylase

Gene Summary

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]

  • phenylalanine-4-hydroxylase
  • phe-4-monooxygenase
  • phenylalanine 4-monooxygenase
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