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. 2018 May 4;31:226–242. doi: 10.1016/j.ebiom.2018.04.024

Fig. 5.

Fig. 5

Dysregulation of the GC-KLF15-BCAA pathway in intermediate SMA mice and prednisolone-induced phenotypic improvements. a. qPCR analysis of Klf15 mRNA in tibialis anterior (TA) of Smn2B/− mice compared to WT animals at different ages (post-natal day (P) 0, P2, P4, P11 and P19). Data represent mean ± SD; n = 4 animals per group; two-way ANOVA; *p < 0.05, **p < 0.01, ***p < 0.001; ns = not significant. b. BCAA metabolism effector genes (mRNA) dysregulated in TAs of pre- and symptomatic Smn2B/− mice compared to WT animals. Data represent fold up- or downregulation with p > 0.05. c. Venn diagram demonstrating the number of upregulated BCAA metabolism effectors in TAs of symptomatic Smn−/−;SMN2 and Smn2B/− mice. d. Weight curves of prednisolone-treated Smn2B/− mice vs. saline-treated animals. Data represent mean ± SD; n = 10–12 animals per group; two-way ANOVA; *p < 0.05; ns = not significant. e. Lifespan of prednisolone-treated Smn2B/− mice vs. saline-treated animals. Data represent Kaplan-Meier curves; n = 10–12 animals per group; Log-rank (Mantel-Cox) test; p < 0.0001. f. Weight curves of Smn2B/+ mice treated with prednisolone or saline. Data represent mean ± SD; n = 7–10 animals per group; two-way ANOVA; ns = not significant.