Neurochemical effects following peripheral administration of tetrahydropterin derivatives to the hph-1 mouse

Journal of Neurochemistry

Volumn 66, Issue 3, 1996, Pages 1150-1156

  Brand, Michael P ^a,b   Hyland, Keith ^c   Engle, Todd ^c   Smith, Isabel ^a   Heales, Simon J R ^b  

^a Institute of Child Health

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c BAYLOR RESEARCH INSTITUTE   (United States)

Author keywords

Deficiency; hph 1 mouse; Tetrahydropterins; Therapy

Indexed keywords

3 HYDROXYBENZYLHYDRAZINE; 3,4 DIHYDROXYPHENYLACETIC ACID; 5 HYDROXYINDOLEACETIC ACID; 6 METHYLTETRAHYDROPTERIN; DOPAMINE; HOMOVANILLIC ACID; SEROTONIN; TETRAHYDROBIOPTERIN; TRYPTOPHAN HYDROXYLASE; TYROSINE 3 MONOOXYGENASE;

ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; DOPAMINE METABOLISM; DOSE TIME EFFECT RELATION; DRUG BLOOD LEVEL; ENZYME ACTIVITY; HYPERPHENYLALANINEMIA; MONOAMINE METABOLISM; MOUSE; NONHUMAN; ORAL DRUG ADMINISTRATION; PRIORITY JOURNAL; SUBCUTANEOUS DRUG ADMINISTRATION;

ANIMALIA;

EID: 0030023052     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1471-4159.1996.66031150.x     Document Type: Article

References (35)

1. Blau N. (1988) Inborn errors of pterm metabolism. Annu. Rev. Nutr. 8, 185-209.
2. Blau N., Thöny B., Heizmann W., and Dhondt J. L. (1993) Tetrahydrobiopterin deficiency: from phenotype to genotype. Pieridines 4, 1-10.
3. Bodor N. and Farag H. (1983) Improved delivery through biological membranes. J. Med. Chem. 26, 313-318.
4. Bolaños J. P., Peuchen S., Heales S. J. R., Land J. M., and Clark J B. (1994) Nitric oxide-mediated inhibition of mitochondrial respiratory chain in cultured astrocytes. J. Neurochem. 63, 910-916.
5. Brand M. P., Heales S. J. R., Land J. M., and Clark J. B. (1995) Tetrahydrobiopterin deficiency and brain nitric oxide synthase deficiency in the hph-1 mouse. J. Inherit. Metab Dis. 18, 33-39.
6. Dhondt J. L. (1991) Strategy for the screening of tetrahydrobiopterin deficiency among hyperphenylalaninaemic patients: 15-years experience. J. Inherit. Metab. Dis. 14, 117-127.
7. Fernstrom J D. and Wurtman R. J. (1972) Brain serotonin content: physiological regulation by plasma neutral amino acids. Science 178, 414-416.
8. Fink J K., Barton N., Cohen W , and Lovenburg W. (1988) Dystonia with marked diurnal variation associated with biopterin deficiency. Neurology 38, 707-711.
9. Frazer A. and Hensler J. G. (1994) Serotonin, in Basic Neurochemistry, 5th edit. (Siegel G J., Agranoff B. W., Albers R W., and Molinoff P. B., eds), pp. 283-308. Raven Press, New York.
10. Howells D. W., Smith I , and Hyland K. (1986) Estimation of tetrahydrobiopterin and other pterins in cerebrospinal fluid using reversed-phase high performance liquid chromatography with electrochemical and fluorescence detection. J. Chromatogr. 381, 285-294.
11. Hyland K. and Bola F. (1989) Tetrahydrobiopterin and biogenic amine status of the hph-1 mouse mutant. Biol. Chem. Hoppe Seyler 370, 387.
12. Hyland K. and Clayton P. T. (1992) Aromatic L-amino acid decarboxylase deficiency: diagnostic methodology. Clin. Chem. 38, 2405-2410.
13. Hyland K. and Engle T. (1993) Tyrosine hydroxylase deficiency in the hph-1 mouse: does tetrahydrobiopterin regulate the steady state concentrations of the aromatic amino acid hydroxylases? (Abstr. 41), in Tenth International Symposium Chemistry and Biology of Pteridines and Folates, p. 63. University of South Alabama, Orange Beach, Alabama.
14. Ichinose H., Ohye T., Takahashi E., Seki N., Hori T., Segawa M., Nomura Y., Endo K., Tanaka H., Tsuji S., Fujita K., and Nagatsu T. (1994) Hereditary progressive dystonia with marked diurnal fluctuation caused by mutations in the GTP cyclohydrolase I gene. Nature Genet. 8, 236-241.
15. Kapatos G. and Kaufman S. (1981) Peripherally administered reduced pterins do enter the brain. Science 212, 955-956.
16. Kaufman S. (1987) Tetrahydrobiopterin and hydroxylation systems in health and disease, in Unconjugated Pterins in Neurobiology: Basic and Clinical Aspects (Lovenburg W. and Levine R. A., eds), pp. 1-28. Taylor and Francis, London.
17. Kaufman S., Kapatos G., McInnes R. R., Schulman J. D., and Rizzo W. B. (1982) Use of tetrahydropterins in the treatment of hyperphenylalaninaemia due to defective synthesis of tetrahydrobiopterin. Pediatrics 70, 376-380.
18. Klatt P., Heinzel B., Mayer B., Ambach E., Werner-Felmayer G., Wachter H., and Werner E. R. (1992) Stimulation of human nitric oxide synthase by tetrahydrobiopterin and selective binding of the cofactor. FEBS Lett 305, 160-162.
19. Koshimura K., Miwa S., and Watanabe Y. (1994) Dopamine-releasing action of 6 R-L-erythro-tetrahydrobiopterin: analysis of its action site using septapterin. J. Neurochem. 63, 649-654.
20. Levine R. A. and Galloway M. P. (1987) The regulation of biogenic amine synthesis and role of tetahydrobiopterin, in Unconjugated Pterins in Neurobiology: Basic and Clinical Aspects (Lovenburg W. and Levine R. A., eds), pp. 119-130. Taylor and Francis, London.
21. Levine R. A., Lovenberg W., Niederwieser A., Leimbacher W., Redweik U., Staudenmann W., and Curtius H Ch. (1983) Penetration of reduced pterins into rat brain: effect on biogenic amine synthesis, in Chemistry and Biology of Pteridines (Blair J., ed), pp. 177-182. Walter de Gruyter, Berlin.
22. Levine R. A., Zoephel G. P., Niederwieser A., Curtius H. Ch., Traub H., and Pfleiderer W. (1985) Tetrahydropterin analogues and their ability to enter the rat striatum after peripheral administration, in Biochemical and Clinical Aspects of Pteridines, Vol. 4 (Wachter H., Curtius H. Ch., and Pfleiderer W., eds), pp. 269-276 Walter de Gruyter, Berlin
23. Levine R. A., Zoephel G. P., Niederwieser A., and Curtius H. Ch. (1987) Entrance of tetrahydropterin derivatives in brain after peripheral administration: effect on biogenic amine metabolism. J. Pharmacol. Exp. Ther. 242, 414-522.
24. Lipton S. A , Choi Y.-B., Pan Z.-H., Lei S. Z., Chen H.-S. V., Sucher N. J., Loscalzo J., Singel D. J., and Stamler J. S. (1993) A redox-based mechanism for the neuroprotective and neurodestructive effects of nitric oxide and related nitroso-compounds. Nature 364, 626-632.
25. McDonald J. D. and Bode V. C. (1988) Hyperphenylalaninaemia in the hph-1 mouse mutant. Pediatr. Res. 23, 63-67.
26. McDonald J. D., Cotton R. G. H., Jennings I., Ledley F. D., Woo S L. C., and Bode V. C. (1988) Biochemical defect of the hph-I mouse mutant is a deficiency in GTP-cyclohydrolase activity. J. Neurochem. 50, 655-657.
27. McInnes R. R., Kaufman S., Warsh J. J., Van Loon G. R., Milstien S., Kapatos G., Soldin S., Walsh P., MacGregor D., and Hanley W. B. (1984) Biopterin synthesis defect. Treatment with L-DOPA and 5-hydroxytryptophan compared with therapy with a tetrahydropterin. J. Clin. Invest. 73, 458-469.
28. Medical Research Council Working Party on Phenylketonuria (1993) Phenylketonuria due to phenylalanine hydroxylase deficiency: an unfolding story. Br. Med. J. 306, 115-119.
29. Murai S., Saito H., Yoshikatsu M., and Itoh T. (1988) Rapid determination of norepinephrine, dopamine, serotonin, their precursor amino acids, and related metabolites in discrete brain areas of mice within ten minutes by HPLC with electrochemical detection. J. Neurochem. 50, 473-479.
30. Okuno S. and Fujisawa H. (1991) Conversion of tyrosine hydroxylase to stable and inactive form by the end products. J. Neurochem. 57, 53-60.
31. Rehse J. and Pfleiderer W. (1989) Synthesis and properties of new 5-substituted tetrahydropterins. Biol. Chem. Hoppe Seyler 370, 390-391.
32. Smith I. and Brenton D. P. (1995) Hyperphenylalaninaemias, in Inborn Metabolic Diseases, Diagnosis and Treatment (Tada K. and Buist N. R. M., ed), pp. 147-160. Springer-Verlag, Heidelberg.
33. Tayeh M. A. and Marietta M. A. (1989) Macrophage oxidation of L-arginine to nitric oxide, nitrite and nitrate. Tetrahydrobiopterin is required as a cofactor. J. Biol. Chem. 264, 19654-19658.
34. Weiner N. and Molinoff P. B. (1994) Catecholamines, in Basic Neurochemistry, 5th edit. (Siegel G. J., Agranoff B. W., Albers R. W., and Molinoff P. B., eds), pp. 261-281. Raven Press, New York.
35. Williams A. C. (1995) Dopamine, dystonia, and the deficient cofactor. Lancet 345, 1130.