Figure 7.

Ighmbp2 ^D564N/D564N mice demonstrate progressive denervation that can be restored with ssAAV9-IGHMBP2. (A) Representative images of the gastrocnemius muscle of PND15 Ighmbp2^+/+ mice (top panels), Ighmbp2^D564N/D564N mice (middle panels) and Ighmbp2^D564N/D564N ssAAV9-IGHMBP2 injected mice (bottom panels). Muscles were labeled with anti-neurofilament heavy polypeptide (NF-H) antibody, anti-synaptic vesicle 2 (SV2) antibody and α-bungarotoxin (α-BTX) that labels the AChRs. Fluorescent images were taken at 40x magnification. (B) Quantification of fully innervated (black), partially innervated (gray) and full denervated (hatched) NMJs in the gastrocnemius muscle. (C) Quantification of fully innervated (black), partially innervated (gray) and full denervated (hatched) NMJs in the EDL muscle. Data analyzed by a two-way ANOVA with Tukey’s post-hoc test for multiple comparisons. Data bars expressed as mean ± SEM. ^****P < 0.0001; n = 3 animals per cohort (n > 100 end plates were analyzed and averaged per cohort); ns = not significant