Fig. 2

CNS-specific expression of LanCL1 transgene significantly prolongs the lifespan, delays disease onset, extends disease progression, and improves motor function of ALS mice. a Breeding strategy for the generation of G93A; LanCL1 cKI triple-transgenic mice and control littermates. Kaplan–Meier curves of the survival showing that LanCL1 overexpression increases median survival b from 141 days for G93A males (n = 15 mice) to 163 days for G93A; LanCL1 cKI males (n = 15 mice), and c from 146 days for G93A females (n = 21 mice) to 168 days for G93A; LanCL1 cKI females (n = 16 mice). **p < 0.01, by log-rank test. Kaplan–Meier curves of disease onset showing that LanCL1 overexpression delays disease onset d from 105 days for G93A males (n = 17 mice) to 119 days for G93A; LanCL1 cKI males (n = 15 mice), and e from 108 days for G93A females (n = 18 mice) to 125.5 days for G93A; LanCL1 cKI females (n = 16 mice). *p < 0.05, by log-rank test. Mean duration of disease (days from onset to endpoint) showing that LanCL1 overexpression extends disease progression f from 30.4 days for G93A males (n = 16 mice) to 43 days for G93A; LanCL1 cKI males (n = 15 mice), and g from 37.5 days for G93A females (n = 17 mice) to 46.3 days for G93A; LanCL1 cKI females (n = 18 mice). **p < 0.01, by two-tailed unpaired Student’s t test. Rotarod tests showing that LanCL1 overexpression improves motor function of male (h) and female (i) G93A mice. Data represent mean ± SEM, n = 11–13 mice per sex per genotype, *p < 0.05, ***p < 0.001, by Mann–Whitney U-tests