title: N-acetyl-L-cysteine improves survival and preserves motor performance in an animal model of familial amyotrophic lateral sclerosis.
abstract: Increasing evidence implicates oxidative damage as a major mechanism in the pathogenesis of amyotrophic lateral sclerosis (ALS). We examined the effect of preventative treatment with N-acetyl-L-cysteine (NAC), an agent that reduces free radical damage, in transgenic mice with a superoxide dismutase (SODI) mutation (G93A), used as an animal model of familial ALS. NAC was administered 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 NAC, an over-the-counter antioxidant, could be explored in clinical trials for ALS.
authors.affiliation: Neurology Service, Massachusetts General Hospital and Harvard Medical School, Boston, USA.
authors.forename: O A
authors.initials: OA
authors.lastname: Andreassen
authors.forename: A
authors.initials: A
authors.lastname: Dedeoglu
authors.forename: P
authors.initials: P
authors.lastname: Klivenyi
authors.forename: M F
authors.initials: MF
authors.lastname: Beal
authors.forename: A I
authors.initials: AI
authors.lastname: Bush
mesh_terms: D000111:Acetylcysteine / Q000494:pharmacology*; D000367:Age Factors; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology / Q000517:prevention & control; D000818:Animals; D004195:Disease Models, Animal; D051379:Mice; D008822:Mice, Transgenic; D009043:Motor Activity / Q000187:drug effects* / Q000502:physiology; D009154:Mutation / Q000502:physiology; D013482:Superoxide Dismutase / Q000235:genetics; D015996:Survival Rate
chemical_list: D013482:Superoxide Dismutase; D000111:Acetylcysteine