6-Pyruvoyltetrahydropterin synthase

Part of the Springer Handbook of Enzymes book series (HDBKENZYMES, volume S7)


Ammonium Sulfate Premature Stop Codon Mutant Enzyme Human Pituitary Gland Salmon Liver 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. [1]
    Thöny, B.; Leimbacher, W.; Blau, N.; Heizmann, C.W.; Burgisser, D.: Human liver 6-pyruvoyl-tetrahydropterin synthase: expression of the cDNA, purification and preliminary characterization of the recombinant protein. Adv. Exp. Med. Biol., 338, 187–190 (1993)PubMedGoogle Scholar
  2. [2]
    Burgisser, D.M.; Thöny, B.; Redweik, U.; Hess, D.; Heizmann, C.W.; Huber, R.; Nar, H.: 6-Pyruvoyl tetrahydropterin synthase, an enzyme with a novel type of active site involving both zinc binding and an intersubunit catalytic triad motif. Site-directed mutagenesis of the proposed active center, characterization of the metal binding site and modeling of substrate binding. J. Mol. Biol., 253, 358–369 (1995)CrossRefPubMedGoogle Scholar
  3. [3]
    Guzman, J.; Redweik, U.; Schoedon, G.; Hunziker, P.; Wiestler, O.D.; Heizmann, C.W.; Blau, N.: Purification and characterization of 6-pyruvoyl tetrahydropterin synthase from human pituitary gland. Enzyme, 46, 287–298 (1992)PubMedGoogle Scholar
  4. [4]
    Takikawa, S.; Curtius, H.Ch.; Redweik, U.; Ghisla, S.: Purification of 6-pyruvoyl-tetrahydropterin synthase from human liver. Biochem. Biophys. Res. Commun., 134, 646–651 (1986)CrossRefPubMedGoogle Scholar
  5. [5]
    Le van, Q.; Katzenmeier, G.; Schwarzkopf, B.; Schmid, C.; Bacher, A.: Biosynthesis of biopterin studies on the mechanism of 6-pyruvoyltetrahydropteridine synthase. Biochem. Biophys. Res. Commun., 151, 512–517 (1988)CrossRefGoogle Scholar
  6. [6]
    Hauer, C.R.; Leimbacher, W.; Hunziker, P.; Neuheiser, F.; Blau, N.; Heizmann, C.W.: 6-Pyruvoyl tetrahydropterin synthase from salmon liver amino acid sequence analysis by tandem mass spectrometry. Biochem. Biophys. Res. Commun., 182, 953–959 (1992)CrossRefPubMedGoogle Scholar
  7. [7]
    Ashida, A.; Hatakeyama, K.; Kagamiyama, H.: cDNA cloning, expression in Escherichia coli and purification of human 6-pyruvoyl-tetrahydropterin synthase. Biochem. Biophys. Res. Commun., 195, 1386–1393 (1993)CrossRefPubMedGoogle Scholar
  8. [8]
    Auerbach, G.; Nar, H.: The pathway from GTP to tetrahydropterin: three-dimensional structures of GTP cyclohydrolase I and 6-pyruvoyl tetrahydropterin synthase. Biol. Chem., 378, 185–192 (1997)PubMedGoogle Scholar
  9. [9]
    Masada, M.: Enzymatic properties of 6-pyruvoyl tetrahydropterin synthase purified from fat bodies of silkworm larvae. Adv. Exp. Med. Biol., 338, 191–194 (1993)PubMedGoogle Scholar
  10. [10]
    Burgisser, D.M.; Thöny, B.; Redweik, U.; Hunziker, P.; Heizmann, C.W.; Blau, N.: Expression and characterization of recombinant human and rat liver 6-pyruvoyl tetrahydropterin synthase. Modified cysteine residues inhibit the enzyme activity. Eur. J. Biochem., 219, 497–502 (1994)CrossRefPubMedGoogle Scholar
  11. [11]
    Oppliger, T.; Thöny, B.; Nar, H.; Burgisser, D.; Huber, R.; Heizmann, C.W.; Blau, N.: Structural and functional consequences of mutations in 6-pyruvoyltetrahydropterin synthase causing hyperphenylalaninemia in humans. J. Biol. Chem., 270, 29498–29506 (1995)CrossRefPubMedGoogle Scholar
  12. [12]
    Inoue, Y.; Kawasaki, Y.; Harada, T.; Hatekeyama, K.; Kagamiyama, H.: Purification and cDNA cloning of rat 6-pyruvoyl-tetrahydropterin synthase. J. Biol. Chem., 266, 20791–20796 (1991)PubMedGoogle Scholar
  13. [13]
    Takikawa, S.; Curtius, H.C.; Redweik, U.; Leimbacher, W.; Ghisla, S.: Biosynthesis of tetrahydropterin. Purification and characterization of 6-pyruvoyl-tetrahydropterin synthase from human liver. Eur. J. Biochem., 161, 295–302 (1986)CrossRefPubMedGoogle Scholar
  14. [14]
    Hasler, T.; Curtius, H.C.: Purification and characterization of 6-pyruvoyl tetrahydropterin synthase from salmon liver. Eur. J. Biochem., 180, 205–211 (1989)CrossRefPubMedGoogle Scholar
  15. [15]
    Park, Y.S.; Kim, J.H.; Jacobson, K.B.; Yim, J.J.: Purification and characterization of 6-pyruvoyl-tetrahydropterin synthase from Drosophila melanogaster. Biochim. Biophys. Acta, 1038, 186–194 (1990)PubMedGoogle Scholar
  16. [16]
    Woo, H.J.; Hwang, Y.K.; Kim, Y.J.; Kang, J.Y.; Choi, Y.K.; Kim, C.G.; Park, Y.S.: Escherichia coli 6-pyruvoyltetrahydropterin synthase ortholog encoded by ygcM has a new catalytic activity for conversion of sepiapterin to 7,8-dihydropterin. FEBS Lett., 523, 234–238 (2002)CrossRefPubMedGoogle Scholar
  17. [17]
    Scherer-Oppliger, T.; Leimbacher, W.; Blau, N.; Thony, B.: Serine 19 of human 6-pyruvoyltetrahydropterin synthase is phosphorylated by cGMP protein kinase II. J. Biol. Chem., 274, 31341–31348 (1999)CrossRefPubMedGoogle Scholar
  18. [18]
    Leitner, K.L.; Meyer, M.; Leimbacher, W.; Peterbauer, A.; Hofer, S.; Heufler, C.; Mueller, A.; Heller, R.; Werner, E.R.; Thoeny, B.; Werner-Felmayer, G.: Low tetrahydrobiopterin biosynthetic capacity of human monocytes is caused by exon skipping in 6-pyruvoyl tetrahydropterin synthase. Biochem. J., 373, 681–688 (2003)CrossRefPubMedGoogle Scholar
  19. [19]
    Woo, H.J.; Kang, J.Y.; Choi, Y.K.; Park, Y.S.: Production of sepiapterin in Escherichia coli by coexpression of cyanobacterial GTP cyclohydrolase I and human 6-pyruvoyltetrahydropterin synthase. Appl. Environ. Microbiol., 68, 3138–3140 (2002)CrossRefPubMedGoogle Scholar
  20. [20]
    Bracher, A.; Eisenreich, W.; Schramek, N.; Ritz, H.; Gotze, E.; Herrmann, A.; Gutlich, M.; Bacher, A.: Biosynthesis of pteridines. NMR studies on the reaction mechanisms of GTP cyclohydrolase I, pyruvoyltetrahydropterin synthase, and sepiapterin reductase. J. Biol. Chem., 273, 28132–28141 (1998)CrossRefPubMedGoogle Scholar
  21. [21]
    Linscheid, P.; Schaffner, A.; Blau, N.; Schoedon, G.: Regulation of 6-pyruvoyltetrahydropterin synthase activity and messenger RNA abundance in human vascular endothelial cells. Circulation, 98, 1703–1706 (1998)PubMedGoogle Scholar
  22. [22]
    Ploom, T.; Thony, B.; Yim, J.; Lee, S.; Nar, H.; Leimbacher, W.; Richardson, J.; Huber, R.; Auerbach, G.: Crystallographic and kinetic investigations on the mechanism of 6-pyruvoyl tetrahydropterin synthase. J. Mol. Biol., 286, 851–860 (1999)CrossRefPubMedGoogle Scholar
  23. [23]
    Liu, T.-T.; Hsiao, K.-J.; Lu, S.-F.; Wu, S.-J.; Wu, K.-F.; Chiang, S.-H.; Liu, X.-Q.; Chen, R.-G.; Yu, W.-M.: Mutation analysis of the 6-pyruvoyl-tetrahydropterin synthase gene in Chinese hyperphenylalaninemia caused by tetrahydrobiopterin synthesis deficiency. Hum. Mutat., 11, 76–83 (1998)CrossRefPubMedGoogle Scholar
  24. [24]
    Tachibana, D.; Fukumasu, H.; Shintaku, H.; Fukumasu, Y.; Yamamasu, S.; Ishiko, O.; Yamano, T.; Ogita, S.: Decreased plasma tetrahydrobiopterin in pregnant women is caused by impaired 6-pyruvoyl tetrahydropterin synthase activity. Int. J. Mol. Med., 9, 49–52 (2002)PubMedGoogle Scholar
  25. [25]
    Elzaouk, L.; Laufs, S.; Heerklotz, D.; Leimbacher, W.; Blau, N.; Resibois, A.; Thoeny, B.: Nuclear localization of tetrahydrobiopterin biosynthetic enzymes. Biochim. Biophys. Acta, 1670, 56–68 (2004)PubMedGoogle Scholar
  26. [26]
    Franscini, N.; Bachli, E.B.; Blau, N.; Fischler, M.; Walter, R.B.; Schaffner, A.; Schoedon, G.: Functional tetrahydrobiopterin synthesis in human platelets. Circulation, 110, 186–192 (2004)CrossRefPubMedGoogle Scholar
  27. [27]
    Iwanaga, N.; Yamamasu, S.-I.; Tachibana, D.; Nishio, J.; Nakai, Y.; Shintaku, H.; Ishiko, O.: Activity of synthetic enzymes of tetrahydrobiopterin in the human placenta. Int. J. Mol. Med., 13, 117–120 (2004)PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2010

Personalised recommendations