EC - 6-pyruvoyltetrahydropterin synthase

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IntEnz Enzyme Nomenclature


Accepted name:
6-pyruvoyltetrahydropterin synthase
Other names:
6-pyruvoyl tetrahydrobiopterin synthase
2-amino-4-oxo-6-[(1S,2R)-1,2-dihydroxy-3-triphosphooxypropyl]-7,8-dihydroxypteridine triphosphate lyase
6-[(1S,2R)-1,2-dihydroxy-3-triphosphooxypropyl]-7,8-dihydroxypteridine triphosphate-lyase (6-pyruvoyl-5,6,7,8-tetrahydropterin-forming)
Systematic name:
7,8-dihydroneopterin 3'-triphosphate triphosphate-lyase (6-pyruvoyl-5,6,7,8-tetrahydropterin-forming)




Catalyses triphosphate elimination and an intramolecular redox reaction in the presence of Mg2+. This enzyme is involved in the de novo synthesis of tetrahydrobiopterin from GTP, with the other enzymes involved being EC (sepiapterin reductase) and EC (GTP cyclohydrolase I) [3].

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , DIAGRAM , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Protein domains and families: PROSITE:PDOC00759
Structural data: CSA , EC2PDB
Gene Ontology: GO:0003874


  1. Milstien, S., Kaufman, S.
    The biosynthesis of tetrahydrobiopterin in rat brain. Purification and characterization of 6-pyruvoyl-tetrahydrobiopterin(2'-oxo) reductase.
    J. Biol. Chem. 264: 8066-8073 (1989). [PMID: 2656673]
  2. Thöny, B., Leimbacher, W., Bürgisser, D., Heinzmann, C.W.
    Human 6-pyruvoyl-tetrahydrobiopterin synthase: cDNA cloning and heterologous expression of the recombinant enzyme.
    Biochem. Biophys. Res. Commun. 189: 1437-1443 (1992). [PMID: 1282802]
  3. Supangat, S., Choi, Y.K., Park, Y.S., Son, D., Han, C.D. and Lee, K.H.
    Expression, purification, crystallization and preliminary X-ray analysis of sepiapterin reductase from Chlorobium tepidum.
    Acta Crystallogr. Sect. F Struct. Biol. Cryst. Commun. 61: 202-204 (2005). [PMID: 16510994]

[EC created 1999 as EC, transferred 2000 to EC, modified 2001]