Figure 4

BAY 55-9837 ameliorates disease phenotype and increases survival of SMA mouse models. SMA∆7 mice were treated daily with intraperitoneal injections of BAY 55-9837 (200.0 μg/kg) from P1 onward. (a) Weights of SMA∆7 mice treated with BAY 55-9837 (black filled square, n =10) or saline (black filled circle, n =10); weights for heterozygous mice treated with saline (black filled triangle, n =5) are also shown for comparison [mean ± SEM (bars)]. (b) Righting times of SMA∆7 mice treated with BAY 55-9837 (black filled square, n = 10) or saline (black filled circle, n =10) [mean ± SEM (bars)]. (c) Kaplan-Meier survival curves of SMA∆7 mice treated with BAY 55-9837 (black filled square, n = 10; median survival 19.5 days) or saline (black filled circle, n =17; median survival 14.0 days ). (d) Kaplan-Meier survival curves of Taiwanese-SMA mice treated with BAY 55-9837 (black filled square, n = 5; median survival 12.0 days) or saline (black filled circle, n =10; median survival 8.0 days); *P < 0.05; ***P < 0.0001, log-rank test.