Abstract
In this study, the effect of tetrahydrobiopterin deficiency on the nitric oxide/cGMP pathway has been investigated in cerebellar slices derived from the hph-1 mouse. This animal displays a partial deficiency of tetrahydrobiopterin. Basal levels of cGMP were significantly reduced (-29.5%) in the hph-1 mouse cerebellum compared to controls. Following kainate stimulation (500 microM) cGMP levels increased in both control and hph-1 preparations but were again significantly lower (-29.1%) in the hph-1 mouse. Exposure of slices to the nitric oxide donors, S-nitroso-N-acetylpenicillamine and S-nitroso-glutathione, revealed no difference in cGMP accumulation between the two groups. These findings suggest that the cerebellar nitric oxide/cGMP pathway may be impaired in partial tetrahydrobiopterin deficiency states due to diminished nitric oxide formation.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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6-Cyano-7-nitroquinoxaline-2,3-dione / pharmacology
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Animals
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Antioxidants / metabolism
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Biopterins / analogs & derivatives
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Biopterins / biosynthesis
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Biopterins / deficiency
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Biopterins / genetics
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Cerebellum / drug effects
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Cerebellum / metabolism*
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Cyclic GMP / metabolism*
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Enzyme Inhibitors / pharmacology
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Excitatory Amino Acid Agonists / pharmacology
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Excitatory Amino Acid Antagonists / pharmacology
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Glutathione / analogs & derivatives
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Glutathione / pharmacology
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Kainic Acid / pharmacology
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Mice
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Mice, Inbred C57BL
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Mice, Inbred CBA
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Mice, Mutant Strains / metabolism*
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Mutation / physiology
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Nitric Oxide / metabolism*
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Nitroarginine / pharmacology
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Nitroso Compounds / pharmacology
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Organ Culture Techniques
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Oxyhemoglobins / pharmacology
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Penicillamine / analogs & derivatives
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Penicillamine / pharmacology
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Platelet Aggregation Inhibitors / pharmacology
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S-Nitroso-N-Acetylpenicillamine
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S-Nitrosoglutathione
Substances
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Antioxidants
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Enzyme Inhibitors
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Excitatory Amino Acid Agonists
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Excitatory Amino Acid Antagonists
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Nitroso Compounds
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Oxyhemoglobins
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Platelet Aggregation Inhibitors
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Nitroarginine
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Biopterins
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Nitric Oxide
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S-Nitrosoglutathione
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6-Cyano-7-nitroquinoxaline-2,3-dione
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S-Nitroso-N-Acetylpenicillamine
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sapropterin
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Glutathione
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Penicillamine
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Cyclic GMP
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Kainic Acid