Tetrahydrobiopterin. An Essential Cofactor for Nitric Oxide Synthases and Amino Acid Hydroxylases.

Nitric Oxide

Volumn , Issue , 2010, Pages 169-209

  Higgins, Christina E ^a   Gross, Steven S ^a  

^a Weill Cornell Medical College of Cornell University   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 84882514143     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1016/B978-0-12-373866-0.00006-X     Document Type: Chapter

References (228)

1. Aktan F. iNOS-mediated nitric oxide production and its regulation. Life Sci. 2004, 75:639-653.
2. Al Sarraj J., Vinson C., Han J., Thiel G. Regulation of GTP cyclohydrolase I gene transcription by basic region leucine zipper transcription factors. J. Cell Biochem. 2005, 96:1003-1020.
3. Anastasiadis P.Z., States J.C., Kuhn D.M., Levine R.A. Co-induction of tetrahydrobiopterin (BH4) levels and tyrosine hydroxylase activity in cultured PC12 cells. Adv. Exp. Med Biol. 1993, 338:227-230.
4. Anastasiadis P.Z., Kuhn D.M., Blitz J., Imerman B.A., Louie M.C., Levine R.A. Regulation of tyrosine hydroxylase and tetrahydrobiopterin biosynthetic enzymes in PC12 cells by NGF, EGF and IFN-gamma. Brain Res. 1996, 713:125-133.
5. Anastasiadis P.Z., States J.C., Imerman B.A., Louie M.C., Kuhn D.M., Levine R.A. Mitogenic effects of tetrahydrobiopterin in PC12 cells. Mol. Pharmacol. 1996, 49:149-155.
6. Anastasiadis P.Z., Bezin L., Imerman B.A., Kuhn D.M., Louie M.C., Levine R.A. Tetrahydrobiopterin as a mediator of PC12 cell proliferation induced by EGF and NGF. Eur. J. Neurosci. 1997, 9:1831-1837.
7. Auerbach G., Herrmann A., Bracher A., Bader G., Gütlich M., Fischer M., Neukamm M., Garrido-Franco M., Richardson J., Nar H., Huber R., Bacher A. Zinc plays a key role in human and bacterial GTP cyclohydrolase I. Proc. Natl. Acad. Sci. U S A 2000, 97:13567-13572.
8. Bader G., Schiffmann S., Herrmann A., Fischer M., Gütlich M., Auerbach G., Ploom T., Bacher A., Huber R., Lemm T. Crystal structure of rat GTP cyclohydrolase I feedback regulatory protein, GFRP. J. Mol. Biol. 2001, 312:1051-1057.
9. Baek K.J., Thiel B.A., Lucas S., Stuehr D.J. Macrophage nitric oxide synthase subunits. Purification, characterization, and role of prosthetic groups and substrate in regulating their association into a dimeric enzyme. J. Biol. Chem. 1993, 268:21120-21129.
10. Balligand J.L., Ungureanu-Longrois D., Simmons W.W., Pimental D., Malinski T.A., Kapturczak M., Taha Z., Lowenstein C.J., Davidoff A.J., Kelly R.A. Cytokine-inducible nitric oxide synthase (iNOS) expression in cardiac myocytes. Characterization and regulation of iNOS expression and detection of iNOS activity in single cardiac myocytes in vitro. J. Biol. Chem. 1994, 269:27580-27588.
11. Bassan A., Blomberg M.R., Siegbahn P.E. Mechanism of dioxygen cleavage in tetrahydrobiopterin-dependent amino acid hydroxylases. Chem. Biodivers. 2003, 9:106-115.
12. Bassan A., Blomberg M.R., Borowski T., Siegbahn P.E. Theoretical studies of enzyme mechanisms involving high-valent iron intermediates. J. Inorg. Biochem. 2006, 100:727-743.
13. Berka V., Tsai A.L. Characterization of interactions among the heme center, tetrahydrobiopterin, and L-arginine binding sites of ferric eNOS using imidazole, cyanide, and nitric oxide as probes. Biochemistry 2000, 39:9373-9383.
14. Blau, N., Beat, T., 2005. Tetrahydrobiopterin, BH4.org. Vol. 2009.
15. Boissel J.P., Bros M., Schröck A., Gödtel-Armbrust U., Förstermann U. Cyclic AMP-mediated upregulation of the expression of neuronal NO synthase in human A673 neuroepithelioma cells results in a decrease in the level of bioactive NO production: analysis of the signaling mechanisms that are involved. Biochemistry 2004, 43:7197-7206.
16. Bonafé L., Thöny B., Penzien J.M., Czarnecki B., Blau N. Mutations in the sepiapterin reductase gene cause a novel tetrahydrobiopterin-dependent monoamine-neurotransmitter deficiency without hyperphenylalaninemia. Am. J. Hum. Genet. 2001, 69:269-277.
17. Bowling K.M., Huang Z., Xu D., Ferdousy F., Funderburk C.D., Karnik N., Neckameyer W., O'Donnell J. Direct binding of GTP cyclohydrolase and tyrosine hydroxylase: Regulatory interactions between key enzymes in dopamine biosynthesis. J. Biol. Chem. 2008, 283:31449-31459.
18. Bracher A., Eisenreich W., Schramek N., Ritz H., Götze E., Herrmann A., Gütlich M., Bacher A. Biosynthesis of pteridines. NMR studies on the reaction mechanisms of GTP cyclohydrolase I, pyruvoyltetrahydropterin synthase, and sepiapterin reductase. J. Biol. Chem. 1998, 273:28132-28141.
19. Bracher A., Fischer M., Eisenreich W., Ritz H., Schramek N., Boyle P., Gentili P., Huber R., Nar H., Auerbach G., Bacher A. Histidine 179 mutants of GTP cyclohydrolase I catalyze the formation of 2-amino-5-formylamino-6-ribofuranosylamino-4(3H)-pyrimidinone triphosphate. J. Biol. Chem. 1999, 274:16727-16735.
20. Bracher A., Schramek N., Bacher A. Biosynthesis of pteridines. Stopped-flow kinetic analysis of GTP cyclohydrolase I. Biochemistry 2001, 40:7896-7902.
21. Brand M.P., Heales S.J., Land J.M., Clark J.B. Tetrahydrobiopterin deficiency and brain nitric oxide synthase in the hph1 mouse. J. Inherit. Metab. Dis. 1995, 18:33-39.
22. Brand M.P., Briddon A., Land J.M., Clark J.B., Heales S.J. Impairment of the nitric oxide/cyclic GMP pathway in cerebellar slices prepared from the hph-1 mouse. Brain Res. 1996, 735:169-172.
23. Bredt D.S., Hwang P.M., Glatt C.E., Lowenstein C., Reed R.R., Snyder S.H. Cloned and expressed nitric oxide synthase structurally resembles cytochrome P-450 reductase. Nature 1991, 351:714-718.
24. Brenneman A.R., Kaufman S. Characteristics of the hepatic phenylalanine-hydroxylating system in newborn rats. J. Biol. Chem. 1965, 240:3617-3622.
25. Bune A.J., Brand M.P., Heales S.J., Shergill J.K., Cammack R., Cook H.T. Inhibition of tetrahydrobiopterin synthesis reduces in vivo nitric oxide production in experimental endotoxic shock. Biochem. Biophys. Res. Commun. 1996, 220:13-19.
26. Burg A.W., Brown G.M. The biosynthesis of folic acid. 8. Purification and properties of the enzyme that catalyzes the production of formate from carbon atom 8 of guanosine triphosphate. J. Biol. Chem. 1968, 243:2349-2358.
27. Chalupsky K., Cai H. Endothelial dihydrofolate reductase: critical for nitric oxide bioavailability and role in angiotensin II uncoupling of endothelial nitric oxide synthase. Proc. Natl Acad. Sci. U.S.A 2005, 102:9056-9061.
28. Channon K.M. Tetrahydrobiopterin: regulator of endothelial nitric oxide synthase in vascular disease. Trends Cardiovasc. Med. 2004, 14:323-327.
29. Chao C.C., Lokensgard J.R., Sheng W.S., Hu S., Peterson P.K. IL-1-induced iNOS expression in human astrocytes via NF-kappa B. Neuroreport 1997, 8:3163-3166.
30. Chavan B., Beazley W., Wood J.M., Rokos H., Ichinose H., Schallreuter K.U. H2O2 increases de novo synthesis of (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin via GTP cyclohydrolase I and its feedback regulatory protein in vitiligo. J. Inherit. Metab. Dis. 2008, 32:86-94.
31. Chen J., Anderson J.B., Deweese-Scott C., Fedorova N.D., Geer L.Y., He S., Hurwitz D.I., Jackson J.D., Jacobs A.R., Lanczycki C.J., Liebert C.A., Liu C., Madej T., Marchler-Bauer A., Marchler G.H., Mazumder R., Nikolskaya A.N., Rao B.S., Panchenko A.R., Shoemaker B.A., Simonyan V., Song J.S., Thiessen P.A., Vasudevan S., Wang Y., Yamashita R.A., Yin J.J., Bryant S.H. MMDB: Entrez's 3D-structure database. Nucleic Acids Res. 2003, 31:474.
32. Choi H.J., Jang Y.J., Kim H.J., Hwang O. Tetrahydrobiopterin is released from and causes preferential death of catecholaminergic cells by oxidative stress. Mol. Pharmacol. 2000, 58:633-640.
33. Colloc'h N., el Hajji M., Bachet B., L'Hermite G., Schiltz M., Prangé T., Castro B., Mornon J.P. Crystal structure of the protein drug urate oxidase-inhibitor complex at 2.05A resolution. Nat. Struct. Biol. 1997, 4:947-952.
34. Colloc'h N., Poupon A., Mornon J. Sequence and structural features of the T-fold, an original tunnelling building unit. Proteins 2000, 39:142-154.
35. Cosentino F., Barker J.E., Brand M.P., Heales S.J., Werner E.R., Tippins J.R., West N., Channon K.M., Volpe M., Lüscher T.F. Reactive oxygen species mediate endothelium-dependent relaxations in tetrahydrobiopterin-deficient mice. Arterioscler.Thromb. Vasc. Biol. 2001, 21:496-502.
36. Crabtree M.J., Smith C.L., Lam G., Goligorsky M.S., Gross S.S. Ratio of 5,6,7,8-tetrahydrobiopterin to 7,8-dihydrobiopterin in endothelial cells determines glucose-elicited changes in NO vs. superoxide production by eNOS. Am. J. Physiol. Heart Circ. Physiol. 2008, 294:H1530-H1540.
37. Craine J.E., Hall E.S., Kaufman S. The isolation and characterization of dihydropteridine reductase from sheep liver. J. Biol. Chem. 1972, 247:6082-6091.
38. Crane B.R., Arvai A.S., Gachhui R., Wu C., Ghosh D.K., Getzoff E.D., Stuehr D.J., Tainer J.A. The structure of nitric oxide synthase oxygenase domain and inhibitor complexes. Science 1997, 278:425.
39. Crane B.R., Arvai A.S., Ghosh D.K., Wu C., Getzoff E.D., Stuehr D.J., Tainer J.A. Structure of nitric oxide synthase oxygenase dimer with pterin and substrate. Science 1998, 279:2121.
40. Crane B.R., Arvai A.S., Ghosh S., Getzoff E.D., Stuehr D.J., Tainer J.A. Structures of the N(omega)-hydroxy-L-arginine complex of inducible nitric oxide synthase oxygenase dimer with active and inactive pterins. Biochemistry 2000, 39:4608-4621.
41. Curtius H.C., Matasovic A., Schoedon G., Kuster T., Guibaud P., Giudici T., Blau N. 7-Substituted pterins. A new class of mammalian pteridines. J. Biol. Chem. 1990, 265:3923-3930.
42. d'Uscio L.V., Milstien S., Richardson D., Smith L., Katusic Z.S. Long-term vitamin C treatment increases vascular tetrahydrobiopterin levels and nitric oxide synthase activity. Circ. Res. 2003, 92:88-95.
43. Danks D. Tetrahydrobiopterin treatment of variant form of phenylketonuria. Lancet 1975, 306:1043.
44. Dassesse D., Hemmens B., Cuvelier L., Résibois A. GTP-cyclohydrolase-I like immunoreactivity in rat brain. Brain Res. 1997, 777:187-201.
45. Du M., Yeh H.C., Berka V., Wang L.H., Tsai A.L. Redox properties of human endothelial nitric-oxide synthase oxygenase and reductase domains purified from yeast expression system. J. Biol. Chem. 2003, 278:6002-6011.
46. Eberhardt W., Kunz D., Pfeilschifter J. Pyrrolidine dithiocarbamate differentially affects interleukin 1 beta- and cAMP-induced nitric oxide synthase expression in rat renal mesangial cells. Biochem. Biophys. Res. Commun. 1994, 200:163-170.
47. Elzaouk L., Laufs S., Heerklotz D., Leimbacher W., Blau N., Résibois A., Thöny B. Nuclear localization of tetrahydrobiopterin biosynthetic enzymes. Biochim. Biophys. Acta. 2004, 1670:56-68.
48. Erlandsen H., Bjørgo E., Flatmark T., Stevens R.C. Crystal structure and site-specific mutagenesis of pterin-bound human phenylalanine hydroxylase. Biochemistry 2000, 39:2208-2217.
49. Fischmann T.O., Hruza A., Niu X.D., Fossetta J.D., Lunn C.A., Dolphin E., Prongay A.J., Reichert P., Lundell D.J., Narula S.K., Weber P.C. Structural characterization of nitric oxide synthase isoforms reveals striking active-site conservation. Nat. Struct. Biol. 1999, 6:233-242.
50. Flatmark T., Stevens R.C. Structural insight into the aromatic amino acid hydroxylases and their disease-related mutant forms. Chem. Rev. 1999, 99:2137-2160.
51. Foxton R., Land J., Heales S.J. Tetrahydrobiopterin availability in Parkinson's and Alzheimer's disease; potential pathogenic mechanisms. Neurochem. Res. 2007, 32:751-756.
52. Funderburk C.D., Bowling K.M., Xu D., Huang Z., O'Donnell J.M. Atypical N-terminal extensions confer novel regulatory properties on GTP cyclohydrolase isoforms in Drosophila melanogaster. J. Biol. Chem. 2006, 281:33302-33312.
53. Furukawa Y., Nygaard T.G., Gütlich M., Rajput A.H., Pifl C., DiStefano L., Chang L.J., Price K., Shimadzu M., Hornykiewicz O., Haycock J.W., Kish S.J. Striatal biopterin and tyrosine hydroxylase protein reduction in dopa-responsive dystonia. Neurology 1999, 53:1032-1041.
54. Ghosh D.K., Wu C., Pitters E., Moloney M., Werner E.R., Mayer B., Stuehr D.J. Characterization of the inducible nitric oxide synthase oxygenase domain identifies a 49 amino acid segment required for subunit dimerization and tetrahydrobiopterin interaction. Biochemistry 1997, 36:10609-10619.
55. Gil M., McKinney C., Lee M.K., Eells J.B., Phyillaier M.A., Nikodem V.M. Regulation of GTP cyclohydrolase I expression by orphan receptor Nurr1 in cell culture and in vivo. J. Neurochem. 2007, 101:142-150.
56. Giovanelli J., Campos K.L., Kaufman S. Tetrahydrobiopterin, a cofactor for rat cerebellar nitric oxide synthase, does not function as a reactant in the oxygenation of arginine. Proc. Natl Acad. Sci. U.S.A 1991, 88:7091-7095.
57. Golderer G., Werner E.R., Heufler C., Strohmaier W., Gröbner P., Werner-Felmayer G. GTP cyclohydrolase I mRNA: novel splice variants in the slime mould Physarum polycephalum and in human monocytes (THP-1) indicate conservation of mRNA processing. Biochem. J. 2001, 355:499.
58. Goodwill K.E., Sabatier C., Stevens R.C. Crystal structure of tyrosine hydroxylase with bound cofactor analogue and iron at 2.05A resolution: self-hydroxylation of Phe300 and the pterin-binding site. Biochemistry 1998, 37:13437-13445.
59. Grenett H.E., Ledley F.D., Reed L.L., Woo S.L. Full-length cDNA for rabbit tryptophan hydroxylase: functional domains and evolution of aromatic amino acid hydroxylases. Proc. Natl Acad. Sci. U.S.A 1987, 84:5530-5534.
60. Grima B., Lamouroux A., Boni C., Julien J.F., Javoy-Agid F., Mallet J. A single human gene encoding multiple tyrosine hydroxylases with different predicted functional characteristics. Nature 1987, 326:707-711.
61. Gross S.S., Jaffe E.A., Levi R., Kilbourn R.G. Cytokine-activated endothelial cells express an isotype of nitric oxide synthase which is tetrahydrobiopterin-dependent, calmodulin-independent and inhibited by arginine analogs with a rank-order of potency characteristic of activated macrophages. Biochem. Biophys. Res. Commun. 1991, 178:823-829.
62. Gross S.S., Levi R. Tetrahydrobiopterin synthesis. An absolute requirement for cytokine-induced nitric oxide generation by vascular smooth muscle. J. Biol. Chem. 1992, 267:25722-25729.
63. Gross S.S., Levi R. Tetrahydrobiopterin synthesis. An absolute requirement for cytokine-induced nitric oxide generation by vascular smooth muscle. J. Biol. Chem. 1992, 267:25722-25729.
64. Gross S.S., Levi R., Madera A., Park K.H., Vane J., Hattori Y. Tetrahydrobiopterin synthesis is induced by LPS in vascular smooth muscle and is rate-limiting for nitric oxide production. Adv. Exp. Med. Biol. 1993, 338:295-300.
65. Gütlich M., Jaeger E., Rücknagel K.P., Werner T., Rödl W., Ziegler I., Bacher A. Human GTP cyclohydrolase I: only one out of three cDNA isoforms gives rise to the active enzyme. Biochem. J. 1994, 302:215-221.
66. Hagenah J., Saunders-Pullman R., Hedrich K., Kabakci K., Habermann K., Wiegers K., Mohrmann K., Lohnau T., Raymond D., Vieregge P., Nygaard T., Ozelius L.J., Bressman S.B., Klein C. High mutation rate in dopa-responsive dystonia: detection with comprehensive GCHI screening. Neurology 2005, 64:908-911.
67. Harada T., Kagamiyama H., Hatakeyama K. Feedback regulation mechanisms for the control of GTP cyclohydrolase I activity. Science 1993, 260:1507-1510.
68. Hatakeyama K., Harada T., Suzuki S., Watanabe Y., Kagamiyama H. Purification and characterization of rat liver Cooperative binding of GTP to the enzyme. J. Biol. Chem. 1989, 264:21660-21664.
69. Hattori Y., Nakanishi N., Kasai K., Shimoda S.I., Gross S.S. Pyrrolidine dithiocarbamate inhibits immunostimulant-induced tetrahydrobiopterin synthesis in rat vascular smooth muscle. Eur. J. Pharmacol. 1996, 296:107-112.
70. Hattori Y., Nakanishi N., Kasai K. Role of nuclear factor kappa B in cytokine-induced nitric oxide and tetrahydrobiopterin synthesis in rat neonatal cardiac myocytes. J. Mol. Cell. Cardiol. 1997, 29:1585-1592.
71. Hegg E.L., Que L. The 2-His-1-carboxylate facial triad--an emerging structural motif in mononuclear non-heme iron(II) enzymes. Eur J Biochem 1997, 250:625-629.
72. Heitzer T., Brockhoff C., Mayer B., Warnholtz A., Mollnau H., Henne S., Meinertz T., Münzel T. Tetrahydrobiopterin improves endothelium-dependent vasodilation in chronic smokers: evidence for a dysfunctional nitric oxide synthase. Circ. Res. 2000, 86:E36-E41.
73. Hesslinger C., Kremmer E., Hültner L., Ueffing M., Ziegler I. Phosphorylation of GTP cyclohydrolase I and modulation of its activity in rodent mast cells. GTP cyclohydrolase I hyperphosphorylation is coupled to high affinity IgE receptor signaling and involves protein kinase C. J. Biol. Chem. 1998, 273:21616-21622.
74. Hevel J.M., Marletta M.A. Macrophage nitric oxide synthase: relationship between enzyme-bound tetrahydrobiopterin and synthase activity. Biochemistry 1992, 31:7160-7165.
75. Hogue C. Cn3D: a new generation of three-dimensional molecular structure viewer. Trends Biochem. Sci. 1997, 22:314-316.
76. Hopkins F. The Pigments of the Pieridæ: A contribution to the study of excretory substances which function in ornament. Phil. Trans. R. Soc. Lond. B 1895, 186:661-682.
77. Hosoda S., Glick D. Studies in Histochemistry. LXXIX. Properties of tryptophan hydroxylase from neoplastic nurine mast cells. J. Biol. Chem. 1966, 241:192.
78. Huang A., Zhang Y.Y., Chen K., Hatakeyama K., Keaney J.F. Cytokine-stimulated GTP cyclohydrolase I expression in endothelial cells requires coordinated activation of nuclear factor-kappaB and Stat1/Stat3. Circ. Res. 2005, 96:164-171.
79. Huber C., Batchelor J.R., Fuchs D., Hausen A., Lang A., Niederwieser D., Reibnegger G., Swetly P., Troppmair J., Wachter H. Immune response-associated production of neopterin. Release from macrophages primarily under control of interferon-gamma. J. Exp. Med. 1984, 160:310-316.
80. Hurshman A.R., Krebs C., Edmondson D.E., Huynh B.H., Marletta M.A. Formation of a pterin radical in the reaction of the heme domain of inducible nitric oxide synthase with oxygen. Biochemistry 1999, 38:15689-15696.
81. Hwu W.L., Wang P.J., Hsiao K.J., Wang T.R., Chiou Y.W., Lee Y.M. Dopa-responsive dystonia induced by a recessive GTP cyclohydrolase I mutation. Hum. Genet. 1999, 105:226-230.
82. Hwu W.L., Chiou Y.W., Lai S.Y., Lee Y.M. Dopa-responsive dystonia is induced by a dominant-negative mechanism. Ann. Neurol. 2000, 48:609-613.
83. Hwu W.L., Yeh H.Y., Fang S.W., Chiang H.S., Chiou Y.W., Lee Y.M. Regulation of GTP cyclohydrolase I by alternative splicing in mononuclear cells. Biochem. Biophys. Res. Commun. 2003, 306:937-942.
84. Hyland K., Gunasekara R.S., Munk-Martin T.L., Arnold L.A., Engle T. The hph-1 mouse: a model for dominantly inherited GTP-cyclohydrolase deficiency. Ann. Neurol. 2003, 54(Suppl 6):S46-S48.
85. Ichinose H., Ohye T., Takahashi E., Seki N., Hori T., Segawa M., Nomura Y., Endo K., Tanaka H., Tsuji S. Hereditary progressive dystonia with marked diurnal fluctuation caused by mutations in the GTP cyclohydrolase I gene. Nat. Genet. 1994, 8:236-242.
86. Iino T., Tabata M., Takikawa S., Sawada H., Shintaku H., Ishikura S., Hara A. Tetrahydrobiopterin is synthesized from 6-pyruvoyl-tetrahydropterin by the human aldo-keto reductase AKR1 family members. Archives of biochemistry and biophysics Arch. Biochem. Biophys. 2003, 416:180-187.
87. Imazumi K., Sasaki T., Takahashi K., Takai Y. Identification of a rabphilin-3A-interacting protein as GTP cyclohydrolase I in PC12 cells. Biochem. Biophys. Res. Commun. 1994, 205:1409-1416.
88. Ionova I.A., Vásquez-Vivar J., Whitsett J., Herrnreiter A., Medhora M., Cooley B.C., Pieper G.M. Deficient BH4 production via de novo and salvage pathways regulates NO responses to cytokines in adult cardiac myocytes. Am. J. Physiol. Heart Circ. Physiol. 2008, 295:H2178-H2187.
89. Iozumi K., Hoganson G.E., Pennella R., Everett M.A., Fuller B.B. Role of tyrosinase as the determinant of pigmentation in cultured human melanocytes. J. Invest. Dermatol. 1993, 100:806-811.
90. Ishida Y. Peripherally administered tetrahydrobiopterin increases in vivo tryptophan hydroxylase activity in the striatum after transplantation of fetal ventral mesencephalon in six hydroxydopamine lesioned rats. Neurosci. Lett. 1998, 253:45-48.
91. Ito T., Suzuki T., Ichinose H. Nerve growth factor-induced expression of the GTP cyclohydrolase I gene via Ras/MEK pathway in PC12D cells. J. Neurochem. 2005, 95:563-569.
92. Iyengar R., Stuehr D.J., Marletta M.A. Macrophage synthesis of nitrite, nitrate, and N-nitrosamines: precursors and role of the respiratory burst. Proc. Natl Acad. Sci. U.S.A 1987, 84:6369-6373.
93. Kaneda N., Kobayashi K., Ichinose H., Kishi F., Nakazawa A., Kurosawa Y., Fujita K., Nagatsu T. Isolation of a novel cDNA clone for human tyrosine hydroxylase: alternative RNA splicing produces four kinds of mRNA from a single gene. Biochem. Biophys. Res. Commun. 1987, 146:971-975.
94. Kapatos G., Hirayama K., Shimoji M., Milstien S. GTP cyclohydrolase I feedback regulatory protein is expressed in serotonin neurons and regulates tetrahydrobiopterin biosynthesis. J. Neurochem. 1999, 72:669-675.
95. Kapatos G., Stegenga S.L., Hirayama K. Identification and characterization of basal and cyclic AMP response elements in the promoter of the rat GTP cyclohydrolase I gene. J. Biol. Chem. 2000, 275:5947-5957.
96. Kappock T.J., Caradonna J.P. Pterin-dependent amino acid hydroxylases. Chem. Rev. 1996, 96:2659-2756.
97. Kaufman S. On the structure of the phenylalanine hydroxylation cofactor. J. Biol. Chem. 1962, 237:2712-2713.
98. Kaufman S. Studies on the structure of the primary oxidation product formed from tetrahydropteridines during phenylalanine hydroxylation. J. Biol. Chem. 1964, 239:332-338.
99. Kaufman S., Holtzman N.A., Milstien S., Butler L.J., Krumholz A. Phenylketonuria due to a deficiency of dihydropteridine reductase. N. Engl. J. Med. 1975, 293:785-790.
100. Kaufman S., Pollock R.J., Summer G.K., Das A.K., Hajra A.K. Dependence of an alkyl glycol-ether monooxygenase activity upon tetrahydropterins. Biochim. Biophys. Acta 1990, 1040:19-27.
101. Kettler R., Bartholini G., Pletscher A. In vivo enhancement of tyrosine hydroxylation in rat striatum by tetrahydrobiopterin. Nature 1974, 249:476-478.
102. Kidder G.W., Dewey V.C. Relationship between pyrimidine and lipid biosynthesis and unconjugated pteridine. Biochem. Biophys. Res. Commun. 1963, 12:280-283.
103. Kinoshita H., Milstien S., Wambi C., Katusic Z.S. Inhibition of tetrahydrobiopterin biosynthesis impairs endothelium-dependent relaxations in canine basilar artery. Am. J. Physiol. 1997, 273:H718-H724.
104. Kirsch M., Korth H., Stenert V., Sustmann R., De Groot H. The autoxidation of tetrahydrobiopterin revisited: Proof of superoxide formation from reaction of tetrahydrobiopterin with molecular oxygen. J. Biol. Chem. 2003, 278:24481.
105. Klatt P., Schmid M., Leopold E., Schmidt K., Werner E.R., Mayer B. The pteridine binding site of brain nitric oxide synthase. Tetrahydrobiopterin binding kinetics, specificity, and allosteric interaction with the substrate domain. J. Biol. Chem. 1994, 269:13861-13866.
106. Klatt P., Schmidt K., Lehner D., Glatter O., Bächinger H.P., Mayer B. Structural analysis of porcine brain nitric oxide synthase reveals a role for tetrahydrobiopterin and L-arginine in the formation of an SDS-resistant dimer. EMBO J 1995, 14:3687-3695.
107. Kobayashi T., Hasegawa H., Kaneko E., Ichiyama A. Gastrointestinal serotonin: depletion due to tetrahydrobiopterin deficiency induced by 2,4-diamino-6-hydroxypyrimidine administration. J. Pharmacol. Exp. Ther. 1991, 256:773-779.
108. Koehntop K., Emerson J., Que L. The 2-His-1-carboxylate facial triad: a versatile platform for dioxygen activation by mononuclear non-heme iron(II) enzymes. J. Biol. Inorg. Chem. 2005, 10:87-93.
109. Komori Y., Hyun J., Chiang K., Fukuto J.M. The role of thiols in the apparent activation of rat brain nitric oxide synthase (NOS). J. Biochem. 1995, 117:923-927.
110. Koshimura K., Miwa S., Lee K., Fujiwara M., Watanabe Y. Enhancement of dopamine release in vivo from the rat striatum by dialytic perfusion of 6R-L-erythro-5,6,7,8-tetrahydrobiopterin. J. Neurochem. 1990, 54:1391-1397.
111. Koshimura K., Miwa S., Watanabe Y. Dopamine-releasing action of 6R-L-erythro-tetrahydrobiopterin: analysis of its action site using sepiapterin. J. Neurochem. 1994, 63:649-654.
112. Koshimura K., Takagi Y., Miwa S., Kido T., Watanabe Y., Murakami Y., Kato Y., Masaki T. Characterization of a dopamine-releasing action of 6R-L-erythro-tetrahydrobiopterin: comparison with a 6S-form. J. Neurochem. 1995, 65:827-830.
113. Kotsonis P., Fröhlich L.G., Shutenko Z.V., Horejsi R., Pfleiderer W., Schmidt H.H. Allosteric regulation of neuronal nitric oxide synthase by tetrahydrobiopterin and suppression of auto-damaging superoxide. Biochem. J. 2000, 346(Pt 3):767-776.
114. Krishnakumar S., Burton D., Rasco J., Chen X., O'Donnell J. Functional interactions betweem GTP cyclohydrolaseI and tyrosine hydroxylase in Drosophila. J. Neurogenet. 2000, 14:1-23.
115. Kwon N.S., Nathan C.F., Stuehr D.J. Reduced biopterin as a cofactor in the generation of nitrogen oxides by murine macrophages. J. Biol. Chem. 1989, 264:20496-20501.
116. Kwon N.S., Nathan C.F., Gilker C., Griffith O.W., Matthews D.E., Stuehr D.J. L-citrulline production from L-arginine by macrophage nitric oxide synthase. The ureido oxygen derives from dioxygen. J. Biol. Chem. 1990, 265:13442-13445.
117. Lapize C., Plüss C., Werner E.R., Huwiler A., Pfeilschifter J. Protein kinase C phosphorylates and activates GTP cyclohydrolase I in rat renal mesangial cells. Biochem. Biophys. Res. Commun. 1998, 251:802-805.
118. Lee S., Ahn C., Park E., Hwang D.S., Yim J. Biochemical characterization of oligomerization of Escherichia coli GTP cyclohydrolase I. J. Biochem. Mol. Biol. 2002, 35:255-261.
119. Levine R.A., Miller L.P., Lovenberg W. Tetrahydrobiopterin in striatum: localization in dopamine nerve terminals and role in catecholamine synthesis. Science 1981, 214:919-921.
120. Li H., Raman C.S., Glaser C.B., Blasko E., Young T.A., Parkinson J.F., Whitlow M., Poulos T.L. Crystal structures of zinc-free and -bound heme domain of human inducible nitric-oxide synthase. Implications for dimer stability and comparison with endothelial nitric-oxide synthase. J. Biol. Chem. 1999, 274:21276-21284.
121. Li H., Shimizu H., Flinspach M., Jamal J., Yang W., Xian M., Cai T., Wen E.Z., Jia Q., Wang P.G., Poulos T.L. The novel binding mode of N-alkyl-N'-hydroxyguanidine to neuronal nitric oxide synthase provides mechanistic insights into NO biosynthesis. Biochemistry 2002, 41:13868-13875.
122. Li S., Armstrong C.M., Bertin N., Ge H., Milstein S., Boxem M., Vidalain P.-O., Han J.-D.J., Chesneau A., Hao T., Goldberg D.S., Li N., Martinez M., Rual J.-F., Lamesch P., Xu L., Tewari M., Wong S.L., Zhang L.V., Berriz G.F., Jacotot L., Vaglio P., Reboul J., Hirozane-Kishikawa T., Li Q., Gabel H.W., Elewa A., Baumgartner B., Rose D.J., Yu H., Bosak S., Sequerra R., Fraser A., Mango S.E., Saxton W.M., Strome S., Van Den Heuvel S., Piano F., Vandenhaute J., Sardet C., Gerstein M., Doucette-Stamm L., Gunsalus K.C., Harper J.W., Cusick M.E., Roth F.P., Hill D.E., Vidal M. A map of the interactome network of the metazoan C. elegans. Science 2004, 303:540-543.
123. Maita N., Okada K., Hatakeyama K., Hakoshima T. Crystal structure of the stimulatory complex of GTP cyclohydrolase I and its feedback regulatory protein GFRP. Proc. Natl Acad. Sci. U.S.A 2002, 99:1212-1217.
124. Maita N., Hatakeyama K., Okada K., Hakoshima T. Structural basis of biopterin-induced inhibition of GTP cyclohydrolase I by GFRP, its feedback regulatory protein. J. Biol. Chem. 2004, 279:51534-51540.
125. Marletta M.A., Yoon P.S., Iyengar R., Leaf C.D., Wishnok J.S. Macrophage oxidation of L-arginine to nitrite and nitrate: nitric oxide is an intermediate. Biochemistry 1988, 27:8706-8711.
126. Martínez A., Knappskog P.M., Haavik J. A structural approach into human tryptophan hydroxylase and its implications for the regulation of serotonin biosynthesis. Curr. Med. Chem. 2001, 8:1077-1091.
127. Masters B.S., McMillan K., Nishimura J., Martasek P., Roman L.J., Sheta E., Gross S.S., Salerno J. Understanding the structural aspects of neuronal nitric oxide synthase (NOS) using microdissection by molecular cloning techniques: molecular dissection of neuronal NOS. Adv. Exp. Med. Biol. 1996, 387:163-169.
128. Masters B.S., McMillan K., Sheta E.A., Nishimura J.S., Roman L.J., Martasek P. Neuronal nitric oxide synthase, a modular enzyme formed by convergent evolution: structure studies of a cysteine thiolate-liganded heme protein that hydroxylates L-arginine to produce NO. as a cellular signal. FASEB J. 1996, 10:552-558.
129. Mataga N., Imamura K., Watanabe Y. 6R-tetrahydrobiopterin perfusion enhances dopamine, serotonin, and glutamate outputs in dialysate from rat striatum and frontal cortex. Brain Res. 1991, 551:64-71.
130. Mayer B., John M., Böhme E. Purification of a Ca2+/calmodulin-dependent nitric oxide synthase from porcine cerebellum. Cofactor-role of tetrahydrobiopterin. FEBS Lett. 1990, 277:215-219.
131. McDonald J.D., Cotton R.G., Jennings I., Ledley F.D., Woo S.L., Bode V.C. Biochemical defect of the hph-1 mouse mutant is a deficiency in GTP-cyclohydrolase activity. J. Neurochem. 1988, 50:655-657.
132. McKean C.M., Schanberg S.M., Giarman N.J. A Mechanism of the indole defect in experimental phenylketonuria. Science 1962, 137:604-605.
133. McTigue M.A., Davies J.F., Kaufman B.T., Kraut J. Crystal structure of chicken liver dihydrofolate reductase complexed with NADP+ and biopterin. Biochemistry 1992, 31:7264-7273.
134. Milstien S., Jaffe H., Kowlessur D., Bonner T. Purification and cloning of the GTP cyclohydrolase I feedback regulatory protein, GFRP. J. Biol. Chem. 1996, 271:19743-19751.
135. Miwa S., Watanabe Y., Hayaishi O. 6R-L-erythro-5,6,7,8-tetrahydrobiopterin as a regulator of dopamine and serotonin biosynthesis in the rat brain. Arch. Biochem. Biophys. 1985, 239:234-241.
136. Moens A.L., Kass D.A. Tetrahydrobiopterin and cardiovascular disease. Arterioscler. Thromb. Vasc. Biol. 2006, 26:2439-2444.
137. Møller N., Meek S., Bigelow M., Andrews J., Nair K.S. The kidney is an important site for in vivo phenylalanine-to-tyrosine conversion in adult humans: A metabolic role of the kidney. Proc. Natl Acad. Sci. U.S.A 2000, 97:1242-1246.
138. Montañez C.S., McDonald J.D. Linkage analysis of the hph-1 mutation and the GTP cyclohydrolase I structural gene. Mol. Genet. Metab. 1999, 68:91-92.
139. Mungrue I.N., Bredt D.S. nNOS at a glance: implications for brain and brawn. J. Cell Sci. 2004, 117:2627.
140. Musacchio J.M., D'Angelo G.L., McQueen C.A. Dihydropteridine reductase: implication on the regulation of catecholamine biosynthesis. Proc. Natl Acad. Sci. U.S.A 1971, 68:2087-2091.
141. Nagatsu T., Levitt M., Udenfriend S. Tyrosine hydroxylase. The initial step in norepinephrine biosynthesis. J. Biol. Chem. 1964, 239:2910-2917.
142. Nagatsu I., Ichinose H., Sakai M., Titani K., Suzuki M., Nagatsu T. Immunocytochemical localization of GTP cyclohydrolase I in the brain, adrenal gland, and liver of mice. J. Neural Transmission 1995, 102:175-188.
143. Nar H., Huber R., Auerbach G., Fischer M., Hösl C., Ritz H., Bracher A., Meining W., Eberhardt S., Bacher A. Active site topology and reaction mechanism of GTP cyclohydrolase I. Proc. Natl Acad. Sci. U.S.A 1995, 92:12120-12125.
144. Nar H., Huber R., Meining W., Schmid C., Weinkauf S., Bacher A. Atomic structure of GTP cyclohydrolase I. Structure 1995, 3:459-466.
145. Narhi L.O., Fulco A.J. Characterization of a catalytically self-sufficient 119,000-dalton cytochrome P-450 monooxygenase induced by barbiturates in Bacillus megaterium. J. Biol. Chem. 1986, 261:7160-7169.
146. Neckameyer W., White K. A single locus encodes both phenylalanine hydroxylase and tryptophan hydroxylase activities in Drosophila. J. Biol. Chem. 1992, 267:4199.
147. Nichol C.A., Lee C.L., Edelstein M.P., Chao J.Y., Duch D.S. Biosynthesis of tetrahydrobiopterin by de novo and salvage pathways in adrenal medulla extracts, mammalian cell cultures, and rat brain in vivo. Proc. Natl Acad. Sci. U.S.A 1983, 80:1546-1550.
148. Ohue T., Koshimura K., Lee K., Watanabe Y., Miwa S. A novel action of 6R-L-erythro-5,6,7,8-tetrahydrobiopterin, a cofactor for hydroxylases of phenylalanine, tyrosine and tryptophan: enhancement of acetylcholine release in vivo in the rat hippocampus. Neurosci. Lett. 1991, 128:93-96.
149. Ota A., Kaneko Y.S., Mori K., Nakashima A., Nagatsu I., Nagatsu T. Effect of peripherally administered lipopolysaccharide (LPS) on GTP cyclohydrolase I, tetrahydrobiopterin and norepinephrine in the locus coeruleus in mice. Stress 2007, 10:131-136.
150. Palmer R.M., Rees D.D., Ashton D.S., Moncada S. L-arginine is the physiological precursor for the formation of nitric oxide in
151. Pannirselvam M., Verma S., Anderson T.J., Triggle C.R. Cellular basis of endothelial dysfunction in small mesenteric arteries from spontaneously diabetic (db/db -/-) mice: role of decreased tetrahydrobiopterin bioavailability. Br. J. Pharmacol. 2002, 136:255-263.
152. Park J.H., Na H.J., Kwon Y.G., Ha K.S., Lee S.J., Kim C.K., Lee K.S., Yoneyama T., Hatakeyama K., Kim P.K., Billiar T.R., Kim Y.M. Nitric oxide (NO) pretreatment increases cytokine-induced NO production in cultured rat hepatocytes by suppressing GTP cyclohydrolase I feedback inhibitory protein level and promoting inducible NO synthase dimerization. J. Biol. Chem. 2002, 277:47073-47079.
153. Patterson E., Broquist H., Albrecht A., von Saltza M., Stokstad E. A new pteridine in urine required for the growth of the protozoon Crithidia fasciculata. J. Am. Chem. Soc. 1955, 77:3167-3168.
154. Peterson T.E., d'Uscio L.V., Cao S., Wang X.L., Katusic Z. Guanosine triphosphate cyclohydrolase I expression and enzymatic activity are present in caveolae of endothelial cells. Hypertension 2009, 53:189-195.
155. Peunova N., Enikolopov G. Nitric oxide triggers a switch to growth arrest during differentiation of neuronal cells. Nature 1995, 375:68-73.
156. Ploom T., Thöny 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. 1999, 286:851-860.
157. Plüss C., Werner E.R., Blau N., Wachter H., Pfeilschifter J. Interleukin 1 beta and cAMP trigger the expression of GTP cyclohydrolase I in rat renal mesangial cells. Biochem. J. 1996, 318:665-671.
158. Presta A., Siddhanta U., Wu C., Sennequier N., Huang L., Abu-Soud H.M., Erzurum S., Stuehr D.J. Comparative functioning of dihydro- and tetrahydropterins in supporting electron transfer, catalysis, and subunit dimerization in inducible nitric oxide synthase. Biochemistry 1998, 37:298-310.
159. Raman C.S., Li H., Martásek P., Král V., Masters B.S., Poulos T.L. Crystal structure of constitutive endothelial nitric oxide synthase: a paradigm for pterin function involving a novel metal center. Cell 1998, 95:939-950.
160. Ramsey A.J., Hillas P.J., Fitzpatrick P.F. Characterization of the active site iron in tyrosine hydroxylase. Redox states of the iron. J. Biol. Chem. 1996, 271:24395-24400.
161. Rebelo J., Auerbach G., Bader G., Bracher A., Nar H., Hösl C., Schramek N., Kaiser J., Bacher A., Huber R., Fischer M. Biosynthesis of pteridines. Reaction mechanism of GTP cyclohydrolase I. J. Mol. Biol. 2003, 326:503-516.
162. Rebrin I., Bailey S.W., Boerth S.R., Ardell M.D., Ayling J.E. Catalytic characterization of 4a-hydroxytetrahydropterin dehydratase. Biochemistry 1995, 34:5801-5810.
163. Rodríguez-Crespo I., Gerber N.C., Ortiz de Montellano P.R. Endothelial nitric-oxide synthase. Expression in Escherichia coli, spectroscopic characterization, and role of tetrahydrobiopterin in dimer formation. J. Biol. Chem. 1996, 271:11462-11467.
164. Schallreuter K.U., Blau N. GTP-cyclohydrolase and vitiligo. Lancet 1997, 350:1254.
165. Schallreuter K.U., Büttner G., Pittelkow M.R., Wood J.M., Swanson N.N., Körner C. Cytotoxicity of 6-biopterin to human melanocytes. Biochem. Biophys. Res. Commun. 1994, 204:43-48.
166. Schallreuter K.U., Wood J.M., Pittelkow M.R., Gütlich M., Lemke K.R., Rödl W., Swanson N.N., Hitzemann K., Ziegler I. Regulation of melanin biosynthesis in the human epidermis by tetrahydrobiopterin. Science 1994, 263:1444-1446.
167. Schallreuter K.U., Wood J.M., Ziegler I., Lemke K.R., Pittelkow M.R., Lindsey N.J., Gütlich M. Defective tetrahydrobiopterin and catecholamine biosynthesis in the depigmentation disorder vitiligo. Biochim. Biophys. Acta 1994, 1226:181-192.
168. Schallreuter K.U., Moore J., Wood J.M., Beazley W.D., Gaze D.C., Tobin D.J., Marshall H.S., Panske A., Panzig E., Hibberts N.A. In vivo and in vitro evidence for hydrogen peroxide (H2O2) accumulation in the epidermis of patients with vitiligo and its successful removal by a UVB-activated pseudocatalase. J. Investig. Dermatol. Symp. Proc. 1999, 4:91-96.
169. Schallreuter K.U., Krüger C., Würfel B.A., Panske A., Wood J.M. From basic research to the bedside: efficacy of topical treatment with pseudocatalase PC-KUS in 71 children with vitiligo. Int. J. Dermatol. 2008, 47:743-753.
170. Scherer-Oppliger T., Matasovic A., Laufs S., Levy H.L., Quackenbush E.J., Blau N., Thöny B. Dominant negative allele (N47D) in a compound heterozygote for a variant of 6-pyruvoyltetrahydropterin synthase deficiency causing transient hyperphenylalaninemia. Hum. Mutat. 1999, 13:286-289.
171. Schmidt P., Lange R., Gorren A., Werner E.R., Mayer B., Andersson K. Formation of a protonated trihydrobiopterin radical cation in the first reaction cycle of neuronal and endothelial nitric oxide synthase detected by electron paramagnetic resonance spectroscopy. J. Biol. Inorg. Chem. 2001, 6:151-158.
172. Schott K., Brand K., Hatakeyama, Kagamiyama H., Maier J., Werner T., Ziegler I. Control of cell-cycle-associated tetrahydrobiopterin synthesis in rat thymocytes. Exp. Cell Res. 1992, 200:105-109.
173. Schramek N., Bracher A., Bacher A. Ring opening is not rate-limiting in the GTP cyclohydrolase I reaction. J. Biol. Chem. 2001, 276:2622-2626.
174. Schramek N., Bracher A., Fischer M., Auerbach G., Nar H., Huber R., Bacher A. Reaction mechanism of GTP cyclohydrolase I: single turnover experiments using a kinetically competent reaction intermediate. J. Mol. Biol. 2002, 316:829-837.
175. Schulz E., Jansen T., Wenzel P., Daiber A., Münzel T. Nitric oxide, tetrahydrobiopterin, oxidative stress, and endothelial dysfunction in hypertension. Antioxid. Redox Signal. 2008, 10:1115-1126.
176. Scott-Burden T., Elizondo E., Ge T., Boulanger C.M., Vanhoutte P.M. Growth factor regulation of interleukin-1 beta-induced nitric oxide synthase and GTP: cyclohydrolase expression in cultured smooth muscle cells. Biochem. Biophys. Res. Commun. 1993, 196:1261-1266.
177. Scriver C.R., Donlon J., Levy H. Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. The Online Metabolic and Molecular Bases of Inherited Disease 2008, McGraw-Hill, New York. D. Valle, A.L. Beaudet, B. Vogelstein, K.W. Kinzler, S.E. Antonarakis, A. Ballabio (Eds.).
178. Searles C.D. Transcriptional and posttranscriptional regulation of endothelial nitric oxide synthase expression. Am. J. Physiol., Cell Physiol. 2006, 291:C803-C816.
179. Shen R., Alam A., Zhang Y. Inhibition of GTP cyclohydrolase I by pterins. Biochim. Biophys. Acta 1988, 965:9-15.
180. Shimizu S., Hiroi T., Ishii M., Hagiwara T., Wajima T., Miyazaki A., Kiuchi Y. Hydrogen peroxide stimulates tetrahydrobiopterin synthesis through activation of the Jak2 tyrosine kinase pathway in vascular endothelial cells. Int. J. Biochem. Cell Biol. 2008, 40:755-765.
181. Shinozaki K., Kashiwagi A., Nishio Y., Okamura T., Yoshida Y., Masada M., Toda N., Kikkawa R. Abnormal biopterin metabolism is a major cause of impaired endothelium-dependent relaxation through nitric oxide/O2- imbalance in insulin-resistant rat aorta. Diabetes 1999, 48:2437-2445.
182. Siddhanta U., Presta A., Fan B., Wolan D., Rousseau D.L., Stuehr D.J. Domain swapping in inducible nitric-oxide synthase. Electron transfer occurs between flavin and heme groups located on adjacent subunits in the dimer. J. Biol. Chem. 1998, 273:18950-18958.
183. Steinmetz M., Plüss C., Christen U., Wolpensinger B., Lustig A., Werner E., Wachter H., Engel A., Aebi U., Pfeilschifter J., Kammerer R. Rat GTP cyclohydrolase I is a homodecameric protein complex containing high-affinity calcium-binding sites. J. Mol Biol. 1998, 279:189-199.
184. Stuehr D.J., Kwon N.S., Nathan C.F., Griffith O.W., Feldman P.L., Wiseman J. N omega-hydroxy-L-arginine is an intermediate in the biosynthesis of nitric oxide from L-arginine. J. Biol. Chem. 1991, 266:6259-6263.
185. Stuehr D.J., Ikeda-Saito M. Spectral characterization of brain and macrophage nitric oxide synthases. Cytochrome P-450-like hemeproteins that contain a flavin semiquinone radical. J. Biol. Chem. 1992, 267:20547-20550.
186. Stuehr D., Pou S., Rosen G.M. Oxygen reduction by nitric-oxide synthases. J. Biol. Chem. 2001, 276:14533-14536.
187. Sumi-Ichinose C., Urano F., Kuroda R., Ohye T., Kojima M., Tazawa M., Shiraishi H., Hagino Y., Nagatsu T., Nomura T., Ichinose H. Catecholamines and serotonin are differently regulated by tetrahydrobiopterin. A study from 6-pyruvoyltetrahydropterin synthase knockout mice. J. Biol. Chem. 2001, 276:41150-41160.
188. Sung Y.J., Hotchkiss J.H., Dietert R.R. 2,4-Diamino-6-hydroxypyrimidine, an inhibitor of GTP cyclohydrolase I, suppresses nitric oxide production by chicken macrophages. Int. J. Immunopharmacol. 1994, 16:101-108.
189. Swick L., Kapatos G. A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions. J. Neurochem. 2006, 97:1447-1455.
190. Takazawa C., Fujimoto K., Homma D., Sumi-Ichinose C., Nomura T., Ichinose H., Katoh S. A brain-specific decrease of the tyrosine hydroxylase protein in sepiapterin reductase-null mice--as a mouse model for Parkinson's disease. Biochem. Biophys. Res. Commun. 2008, 367:787-792.
191. Talwar H.S., Griffiths C.E., Fisher G.J., Russman A., Krach K., Benrazavi S., Voorhees J.J. Differential regulation of tyrosinase activity in skin of white and black individuals in vivo by topical retinoic acid. J. Invest. Dermatol. 1993, 100:800-805.
192. Tanaka Y., Nakagawa N., Kuramitsu S., Yokoyama S., Masui R. Novel reaction mechanism of High-resolution X-ray crystallography of Thermus thermophilus HB8 enzyme complexed with a transition state analogue, the 8-oxoguanine derivative. J. Biochem. 2005, 138:263-275.
193. Tayeh M.A., Marletta M.A. Macrophage oxidation of L-arginine to nitric oxide, nitrite, and nitrate. Tetrahydrobiopterin is required as a cofactor. J. Biol. Chem. 1989, 264:19654-19658.
194. Thöny B., Blau N. Mutations in the BH4-metabolizing genes GTP cyclohydrolase I, 6-pyruvoyl-tetrahydropterin synthase, sepiapterin reductase, carbinolamine-4a-dehydratase, and dihydropteridine reductase. Hum. Mutat. 2006, 27:870-878.
195. Thöny B., Leimbacher W., Blau N., Harvie A., Heizmann C.W. Hyperphenylalaninemia due to defects in tetrahydrobiopterin metabolism: molecular characterization of mutations in 6-pyruvoyl-tetrahydropterin synthase. Am. J. Hum. Genet. 1994, 54:782-792.
196. Thöny B., Neuheiser F., Kierat L., Rolland M.O., Guibaud P., Schlüter T., Germann R., Heidenreich R.A., Duran M., de Klerk J.B., Ayling J.E., Blau N. Mutations in the pterin-4alpha-carbinolamine dehydratase (PCBD) gene cause a benign form of hyperphenylalaninemia. Hum. Genet. 1998, 103:162-167.
197. Thöny B., Calvo A.C., Scherer T., Svebak R.M., Haavik J., Blau N., Martinez A. Tetrahydrobiopterin shows chaperone activity for tyrosine hydroxylase. J. Neurochem. 2008, 106:672-681.
198. Tóth M., Kukor Z., Valent S. Chemical stabilization of tetrahydrobiopterin by L-ascorbic acid: contribution to placental endothelial nitric oxide synthase activity. Mol. Hum. Reprod. 2002, 8:271-280.
199. Ueda S., Matsuoka H., Miyazaki H., Usui M., Okuda S., Imaizumi T. Tetrahydrobiopterin restores endothelial function in long-term smokers. J. Am. Coll. Cardiol. 2000, 35:71-75.
200. Vásquez-Vivar J., Kalyanaraman B., Martásek P., Hogg N., Masters B.S., Karoui H., Tordo P., Pritchard K.A. Superoxide generation by endothelial nitric oxide synthase: the influence of cofactors. Proc. Natl Acad. Sci. U.S.A 1998, 95:9220-9225.
201. Wallick D.E., Bloom L.M., Gaffney B.J., Benkovic S.J. Reductive activation of phenylalanine hydroxylase and its effect on the redox state of the non-heme iron. Biochemistry 1984, 23:1295-1302.
202. Wang J., Stuehr D.J., Ikeda-Saito M., Rousseau D.L. Heme coordination and structure of the catalytic site in nitric oxide synthase. J. Biol. Chem. 1993, 268:22255-22258.
203. Wang Z.Q., Wei C.C., Ghosh S., Meade A.L., Hemann C., Hille R., Stuehr D.J. A conserved tryptophan in nitric oxide synthase regulates heme-dioxy reduction by tetrahydrobiopterin. Biochemistry 2001, 40:12819-12825.
204. Wang L., Erlandsen H., Haavik J., Knappskog P.M., Stevens R.C. Three-dimensional structure of human tryptophan hydroxylase and its implications for the biosynthesis of the neurotransmitters serotonin and melatonin. Biochemistry 2002, 41:12569-12574.
205. Wang Z., Wei C.C., Santolini J., Panda K., Wang Q., Stuehr D.J. A tryptophan that modulates tetrahydrobiopterin-dependent electron transfer in nitric oxide synthase regulates enzyme catalysis by additional mechanisms. Biochemistry 2005, 44:4676-4690.
206. Wang Y., Addess K.J., Chen J., Geer L.Y., He J., He S., Lu S., Madej T., Marchler-Bauer A., Thiessen P.A., Zhang N., Bryant S.H. MMDB: annotating protein sequences with Entrez's 3D-structure database. Nucleic Acids Res 2007, 35:D298-D300.
207. Wang W., Zolty E., Falk S., Summer S., Zhou Z., Gengaro P., Faubel S., Alp N., Channon K., Schrier R. Endotoxemia-related acute kidney injury in transgenic mice with endothelial overexpression of GTP cyclohydrolase-1. Am. J. Physiol., Renal Physiol. 2008, 294:F571-F576.
208. Wei C.C., Wang Z.Q., Wang Q., Meade A.L., Hemann C., Hille R., Stuehr D.J. Rapid kinetic studies link tetrahydrobiopterin radical formation to heme-dioxy reduction and arginine hydroxylation in inducible nitric-oxide synthase. J. Biol. Chem. 2001, 276:315-319.
209. Wei C.C., Wang Z., Hemann C., Hille R., Stuehr D.J. A tetrahydrobiopterin radical forms and then becomes reduced during Nomega-hydroxyarginine oxidation by nitric-oxide synthase. J. Biol. Chem. 2003, 278:46668-46673.
210. Wei C.C., Wang Z., Tejero J., Yang Y., Hemann C., Hille R., Stuehr D.J. Catalytic reduction of a tetrahydrobiopterin radical within nitric-oxide synthase. J. Biol Chem. 2008, 283:11734.
211. Werner E.R., Werner-Felmayer G., Fuchs D., Hausen A., Reibnegger G., Yim J.J., Wachter H. Impact of tumour necrosis factor-alpha and interferon-gamma on tetrahydrobiopterin synthesis in murine fibroblasts and macrophages. Biochem. J. 1991, 280:709-714.
212. Werner E.R., Pitters E., Schmidt K., Wachter H., Werner-Felmayer G., Mayer B. Identification of the 4-amino analogue of tetrahydrobiopterin as a dihydropteridine reductase inhibitor and a potent pteridine antagonist of rat neuronal nitric oxide synthase. Biochem. J. 1996, 320:193-196.
213. Werner-Felmayer G., Prast H., Werner E.R., Philippu A., Wachter H. Induction of GTP cyclohydrolase I by bacterial lipopolysaccharide in the rat. FEBS Lett 1993, 322:223-226.
214. Whitsett J., Picklo M.J., Vasquez-Vivar J. 4-Hydroxy-2-nonenal increases superoxide anion radicalin endothelial cells via stimulated GTP cyclohydrolase proteasomal degradation. Arterioscler. Thromb. Vasc. Biol. 2007, 27:2340-2347.
215. Widder J.D., Chen W., Li L., Dikalov S., Thöny B., Hatakeyama K., Harrison D.G. Regulation of tetrahydrobiopterin biosynthesis by shear stress. Circ. Res. 2007, 101:830-838.
216. Witter K., Werner T., Blusch J.H., Schneider E.M., Riess O., Ziegler I., Rödl W., Bacher A., Gütlich M. Cloning, sequencing and functional studies of the gene encoding human GTP cyclohydrolase I. Gene 1996, 171:285-290.
217. Wood J.M., Schallreuter-Wood K.U., Lindsey N.J., Callaghan S., Gardner M.L. A specific tetrahydrobiopterin binding domain on tyrosinase controls melanogenesis. Biochem. Biophys. Res. Commun. 1995, 206:480-485.
218. Woody R., Brewster M., Glasier C. Progressive intracranial calcification in dihydropteridine reductase deficiency prior to folinic acid therapy. Neurology 1989, 39:673-675.
219. Wyss C.A., Koepfli P., Namdar M., Siegrist P.T., Luscher T.F., Camici P.G., Kaufmann P.A. Tetrahydrobiopterin restores impaired coronary microvascular dysfunction in hypercholesterolaemia. Eur. J. Nucl. Med. Mol. Imaging 2005, 32:84-91.
220. Xu J., Wu Y., Song P., Zhang M., Wang S., Zou M.-H. Proteasome-dependent degradation of guanosine 5'-triphosphate cyclohydrolase I causes tetrahydrobiopterin deficiency in diabetes mellitus. Circulation 2007, 116:944-953.
221. Yang S., Lee Y.J., Kim J.M., Park S., Peris J., Laipis P., Park Y.S., Chung J.H., Oh S.P. A murine model for human sepiapterin-reductase deficiency. Am. J. Hum. Genet. 2006, 78:575-587.
222. Yim J.J., Brown G.M. Characteristics of guanosine triphosphate cyclohydrolase I purified from Escherichia coli. J. Biol. Chem. 1976, 251:5087-5094.
223. Yoneyama T., Brewer J.M., Hatakeyama K. GTP cyclohydrolase I feedback regulatory protein is a pentamer of identical subunits. Purification, cDNA cloning, and bacterial expression. J. Biol. Chem. 1997, 272:9690-9696.
224. Yoneyama T., Hatakeyama K. Decameric GTP cyclohydrolase I forms complexes with two pentameric GTP cyclohydrolase I feedback regulatory proteins in the presence of phenylalanine or of a combination of tetrahydrobiopterin and GTP. J. Biol. Chem. 1998, 273:20102-20108.
225. Yoneyama T., Hatakeyama K. Ligand binding to the inhibitory and stimulatory GTP cyclohydrolase I/GTP cyclohydrolase I feedback regulatory protein complexes. Protein Sci. 2001, 10:871-878.
226. Yoneyama T., Wilson L.M., Hatakeyama K. GTP cyclohydrolase I feedback regulatory protein-dependent and -independent inhibitors of GTP cyclohydrolase I. Arch. Biochem. Biophys. 2001, 388:67-73.
227. Yuwiler A., Louttit R.T. Effects of phenylalanine diet on brain serotonin in the rat. Science 1961, 134:831-832.
228. Zurflüh M.R., Zschocke J., Lindner M., Feillet F., Chery C., Burlina A., Stevens R.C., Thöny B., Blau N. Molecular genetics of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency. Hum. Mutat. 2008, 29:167-175.