N-acetyl-L-cysteine improves survival and preserves motor performance in an animal model of familial amyotrophic lateral sclerosis

Increasing evidence implicates oxidative damage as a major mechanism in the pathogenesis of amyotrophic lateral sclerosis (ALS). We examined the effec t of preventative treatment with N-acetyl-L-cysteine (NAC), an agent that r educes free radical damage. in transgenic mice with a superoxide dismutase (SODI) mutation (G93A), used as an animal model of familial ALS. NAC was ad ministered at 1% concentration in the drinking water from 4-5 weeks of age. The treatment caused a significantly prolonged survival and delayed onset of motor impairment in G93A mice treated with NAC compared to control mice. These results provide further evidence for the involvement of free radical damage in the G93A mice, and support the possibility that NAG, an over-the -counter antioxidant, could be explored in clinical trials for ALS. NeuroRe port 11:2491-2493 (C) 2000 Lippincott Williams & Wilkins.