MitoSOD1 prevents denervation at the neuromuscular junction and rescues the Sod1−/− phenotype. (A) Representative images of neuromuscular junctions from tibialis anterior at 12 months. Numerous intermediate (asterisks) and denervated (arrowheads) endplates are seen in Sod1−/− mice, but are rarely observed in control animals or in mitoSOD1,Sod1−/− mice (scale bar = 50 µm). (B) Quantitative comparison of neuromuscular junction innervation at 4, 8 and 12 months of age. No significant difference was seen between mitoSOD1,Sod1−/−, Sod1+/+ and mitoSOD1,Sod1+/+ mice at any time point. Endplates (964 ± 105) were assessed per muscle (***P < 0.001 for Sod1−/− versus all other genotypes; ANOVA with Tukey post hoc test; n = 3 mice per group). (C) Latency to fall on grip strength testing at 12 months of age (*P < 0.05; ANOVA with Tukey post hoc test; n = 3–4 per group).