Figure 1. Reducing levels of ataxin 2 extends lifespan, improves motor function, and slows the rate of disease progression in TDP-43 transgenic mice.

a,b) Kaplan-Meier survival curves comparing survival of TDP-43^Tg/TgAtxn2^+/+ (n = 28), TDP-43^Tg/TgAtxn2^+/− (n = 20) and TDP-43^Tg/TgAtxn2^−/− (n = 18) mice. Curves were compared by log rank test, and effect size was estimated by a Cox proportional hazards model (HR = hazard ratio). c) The gait impairment score of TDP-43^Tg/Tg mice worsened with age, but this was attenuated by reducing ataxin 2. d) A line was fit to each mouse’s gait scores over time and the slope of that line was plotted, grouping animals by genotype. The error bars are min to max, and the three groups were compared by ordinary one-way ANOVA. e,f) Tremor and kyphosis scores were also decreased by lowering ataxin 2 in TDP-43^Tg/Tg mice. c,e,f) At each age, the three genotypes were compared by ordinary one-way ANOVA. Means are plotted, and error bars indicate S.E.M. *p < .05, **p < .01, ***p < .001.