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. 2019 Jun 20;105(1):221–230. doi: 10.1016/j.ajhg.2019.05.008

Figure 4.

Figure 4

Electrophysiological, Structural, and Motoric Analysis of Mice Injected with SMN-ASO at P1 and Ctrl-ASO or Ncald-ASO3 at P2

(A) Dot plots of CMAP and MUNE values at P21 in HET and SMA mice with mean ± SD. Animals used for CMAP: HET Ctrl-ASO n = 12; HET Ncald-ASO3 n = 13; SMA Ctrl-ASO n = 12; SMA Ncald-ASO3 n = 13. MUNE: HET Ctrl-ASO n = 11; HET Ncald-ASO3 n = 11; SMA Ctrl-ASO n = 9; SMA Ncald-ASO3 n = 9.

(B) Representative NMJ images in HET and SMA animals at P21 showing postsynaptic NMJ region stained with bungarotoxin (BTX, magenta) and presynaptic nerve with neurofilament (NF, green). Scale bar: 10 μm. Graphs below the images show single dot plot values of NMJ areas of all grouped animals with mean ± SD and quantification of maturity of NMJs with mean values of mice per group ± SD. Statistics was performed with mean values of animals per group. n = 4 animals per group, 30–55 NMJs per animal.

(C) Representative pictures and quantifications of hematoxylin and eosin-stained gastrocnemius (GC) muscle fibers from HET and SMA animals at P21. Scale bar: 50 μm. Graphs on the right show single dot plot values of GC areas of all grouped animals with mean ± SD (n = 4 animals per genotype, n = 50 fibers/mouse). For visualization, muscle fibers were grouped according to the area intervals of 100 μm2.

(D) Dot plot of grip strength in HET and SMA animals at 3 months. Single values of each animal are shown as mean ± SD. Number of animals used are: HET Ctrl-ASO n = 14, HET Ncald-ASO3 n = 9, SMA Ctrl-ASO n = 10, SMA Ncald-ASO3 n = 10.

Color legend for all graphs is displayed in (A). For all analyses, ordinary one-way ANOVA with Tukey posthoc test for multiple comparisons was applied. n.s., not significant, ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001.