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.