Protein Name 6-pyruvoyltetrahydropterin synthase
Organism Homo sapiens
Gene ID 5805
Gene Symbol


UniProt Q03393 (PTPS_HUMAN)
Relationships Total Number of functionally related compound(s) : 121
Total Number of Articles : 174

6-pyruvoyltetrahydropterin synthase

Gene Summary

The enzyme encoded by this gene catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity. Mutations in this gene result in hyperphenylalaninemia. [provided by RefSeq, Oct 2008]

  • 6-pyruvoyl tetrahydrobiopterin synthase
  • PTP synthase
  • PTPS
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