Figure 8. scAAV9-Abt1 delivery reduces NMJ denervation in FVB-nmd mutant mice.

Tissue from P12 WT (+/+), Ighmbp2^nmd/nmd, and Ighmbp2^nmd/nmd mice injected with scAAV9-Abt1 (4 × 10¹¹ viral genomes) was evaluated. (A) Quantification of the percentage of end plates of gastrocnemius neuromuscular junctions (NMJs). Assessment of fully denervated (dark gray), partially innervated (light gray), or fully innervated (black) end plates. Statistical significance was determined by 2-way ANOVA with a Tukey’s multiple-comparison post hoc test; ****P ≤ 0.0001. Greater than 100 end plates per cohort were counted and averaged. Data points on graphs represent the average per animal with statistical analysis comparing the average of each animal. (B) Quantification of the percentage of end plates of tibialis anterior NMJs. Assessment of fully denervated (dark gray), partially innervated (light gray), or fully innervated (black) end plates. Statistical significance was determined by 2-way ANOVA with a Tukey’s multiple-comparison post hoc test; *P ≤ 0.0331, ****P ≤ 0.0001. Greater than 100 end plates per cohort were counted and averaged. Data points on graphs represent the average per animal with statistical analysis comparing the average of each animal. (C) Microscopic images of gastrocnemius tissue sections. (D) Microscopic images of tibialis anterior tissue sections. Tissue was assessed by neurofilament heavy chain (NF-H) and synaptic vesicle type 2 (SV2) to label axons and synaptic terminals. bungarotoxin labeled acetylcholine receptors (AChRs). Original magnification, 40×. Analyses were performed with GraphPad Prism software. Data are shown as mean ± SEM. Data points on graph represent the average per animal with statistical analysis comparing the average of each animal. Scale bars: 50 μm.