Figure 3.

I.c.v. delivery of AAV9-IGHMBP2 in high dose repaired the gastrocnemius muscle fibers of early-treated mice more efficiently than late-treated mice

(A) Laminin immuno-stained cross sections of gastrocnemius muscles of high-dose-treated mice at P2 and P8 (n = 4) were examined under 40× magnification and compared to age-matched WT littermates (P42) (n = 3), while untreated FVB-nmd sections (n = 5) were evaluated in comparison to the age-matched HET mice (P14) (n = 3). (B and C) The area and perimeter of gastrocnemius muscle fibers in untreated FVB-nmd mice are drastically smaller than their HET counterparts (one-way ANOVA, p < 0.0001). The area and perimeter of P2-treated gastrocnemius muscle fibers are significantly larger than P8-treated mice (p < 0.0001) but markedly smaller than the age-matched WT cohort (p < 0.0001). (D and E) The area and perimeter of diaphragm muscle fibers in untreated FVB-nmd mice are significantly smaller than their HET counterparts (one-way ANOVA, p < 0.0001). The area and perimeter of P2-treated diaphragm muscle fibers are significantly larger than P8-treated (p < 0.0001) but are not rescued to age-matched WT size (p < 0.0001). Scale bar, 30 μm. Error bars represent mean ± SEM. ∗∗∗∗p < 0.0001.