Degeneration of NO-synthesizing cerebrocortical neurons in transgenic mice expressing mutated superoxide dismutase is not due to elevated nitric oxide levels.
Nitric oxide (NO) synthase (NOS)-containing cerebrocortical neurons degenerate in patients with amyotrophic lateral sclerosis (ALS) and dementia, and in transgenic mice expressing a mutated superoxide dismutase gene (G93A) associated with familial ALS. The cerebral cortex of transgenic mice displayed decreased NOS activity (p<0.001) and cGMP levels (p<0.01), but no changes in NOS content indicating that less NO is produced. Therefore, NOSN degeneration is not caused by elevated NO.
Aguila
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MC
Research and Neurology Services, Department of Veterans Affairs Medical Center and Division of Behavioral Neurology, University of Miami, FL 33136-2115, USA. maguila@mednet.med.miami.edu
Kuljis
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RO
D019943:Amino Acid Substitution; D000818:Animals; D015153:Blotting, Western; D002531:Cerebellum / Q000201:enzymology / Q000378:metabolism / Q000473:pathology; D002540:Cerebral Cortex / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology; D006152:Cyclic GMP / Q000378:metabolism; D004195:Disease Models, Animal; D005838:Genotype; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009154:Mutation; D009410:Nerve Degeneration / Q000235:genetics / Q000378:metabolism*; D009474:Neurons / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology; D009569:Nitric Oxide / Q000096:biosynthesis*; D019001:Nitric Oxide Synthase / Q000378:metabolism; D052248:Nitric Oxide Synthase Type I; D016133:Polymerase Chain Reaction; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism
D009569:Nitric Oxide; D019001:Nitric Oxide Synthase; D052248:Nitric Oxide Synthase Type I; C496321:Nos1 protein, mouse; D013482:Superoxide Dismutase; D006152:Cyclic GMP