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. 2023 Jun 26;13:10374. doi: 10.1038/s41598-023-37496-0

Figure 4.

Figure 4

Effects of AR42 and AR19 on the lifespan and disease end-stage in SMNΔ7 SMA mice. (A, B) SMNΔ7 SMA mice were treated daily with AR42 (5 mg/kg/d; red dashed line) or AR19 (5 mg/kg/d; blue dotted line) beginning at PND04 and monitored for changes in lifespan (A) and disease end-stage, which is defined as the onset of body mass loss (B). AR42 increased survival by 27% (A; p = 0.011; Kaplan–Meier analysis with Mantel–Cox log rank post hoc test) and delayed the onset of body mass loss by 31% (B; p = 0.002; Kaplan–Meier analysis with Mantel–Cox log rank post hoc test). AR19, on the other hand did not affect lifespan or disease end-stage in SMNΔ7 SMA mice. (C, D) SMNΔ7 SMA mice were treated with AR42 (5 mg/kg/d) daily beginning at either PND04 (red dashed line) or PND09 (blue dotted line). Post-symptomatic—i.e. beginning at PND09, or after onset of motor neuron loss—treatment with AR42 did not positively affect survival (C) or onset of body mass loss (D) in these mice. In fact, post-symptomatic AR42 treatment resulted in earlier death of SMNΔ7 mice than vehicle (black line).