QUANTITATIVE METER ASSESSMENT IN FALS MICE - A LONGITUDINAL-STUDY

WE have evaluated the G1H line of transgenic mice overexpressing a fam ilial ALS mutation of SOD1 (Gly-93-->Ala) in tasks assessing different aspects of motor function to determine how early these deficits could be detected and their order of appearance. The earliest deficits were observed in tests of muscle strength and coordination as early as 8 w eeks of age and their development appeared to be biphasic, whereas spo ntaneous activity was not impaired until 15 weeks of age. These studie s show that, in addition to the previously demonstrated histological a nd electromyographic deficits, this transgenic mouse also presents cha nges in motor function reminiscent of the human disease, reinforcing a nd extending its validity as an animal model of familial amyotrophic l ateral sclerosis (FALS) and allowing the investigation of novel drug t reatment for ALS.