Fig. 7: Effects of IL-4 gene therapy on the phenotype, disease onset and life span of SOD1G93A mice.
a G93A-IL-4-LV mice displayed higher body weight than G93A-GFP-LV mice. G93A-IL-4-LV maintained an overall significant higher weight until the day of the sacrifice (each symbol represents the mean ± S.E.M., n = 9 for each group). Linear regression analysis shows that the elevation between the two curves is significant, p < 0.01. b Clinical onset of the disease in G93A-GFP-LV mice (n = 18) and G93A-IL-4-LV mice (n = 10). c Kaplan-Meyer of survival of G93A-GFP-LV mice (n = 18, median survival 131 days) and G93A-IL-4-LV mice (n = 14, median survival 129 days). d Rota-rod tests detected increased time remained on rota-rod in G93A-IL-4-LV mice. Untreated WT mice (n = 5), G93A-GFP-LV mice (n = 5) and G93A-IL-4-LV mice (n = 4). Mice were placed on an accelerated rod with speed from 4 to 40 rpm over 15 min. The latency to fall off the rota-rod was recorded at day 80, 90, and 110, and means ± S.E.M. plotted in the histogram. e–g Immunohistochemistry for ChAt in untreated WT mice, G93A-GFP-LV mice and G93A-IL-4-LV mice at day 100. h Quantifications of ChAt+ neurons scored in the ventral horn of the lumbar spinal cord at day 100 (n = 3 for each group). i–n Real time PCR for Cdk1, CxcR2, P2ry13, Csf1R, Selplg, and CD14 on microglia from E12.5 (n = 3), P2 (n = 3), GFP-LV (n = 4), IL-4-LV (n = 4), G93A-GFP-LV (n = 5) and G93A-IL-4-LV (n = 6). Data are represented as mean ± S.E.M. Data in (b) has been analyzed by Mann–Whitney test, while Log-rank (Mantel–Cox) Test was used to analyze Kaplan–Meyer data of panel (c). Data of panels (d) have been analyzed by Kruskal–Wallis test followed by Dunn’s Multiple Comparison Test. Unpaired t test has been used to analyze data plotted on panel (h). One way ANOVA followed by Tukey’s Multiple Comparison Test has been used to analyze data in panels (i–n). *p < 0.05; **p < 0.01; ***p < 0.001. Scale bar 100 µm
