Figure 8

Flunarizine mitigates the defects at the neuromuscular junctions of SMA mice. (A) Immunofluorescent analyses of the neuromuscular junctions (NMJs) in the tibialis using anti-SNAP25 and anti-neurofilament light chain antibodies in red and labeled alpha-bungarotoxin (BGTX) staining for the acetylcholine receptors (AChRs) in green for the pre- and postsynaptic NMJs, respectively. Scale bar, 100 μm. (B) Frequency histograms depict the proportion of neuromuscular junctions in three stages (plaques, perforated structures and pretzels) in three muscles, namely soleus, plantaris and tibialis. The analysis shows that endplates are more elaborated in the soleus and tibialis of SMA mice upon the flunarizine treatment. Statistical test is two-way ANOVA followed by Tukey’s multiple comparisons test. Three mice per group. (C) Representative motor endplates from the tibialis of controls and SMA mice. Scale bar, 15 μm. (D) Quantification of the areas of the AChR clusters reveals a significant increase in endplate size in control and SMA mice upon flunarizine treatment. Kruskal-Wallis followed by Dunn’s multiple comparison rank test. Wiskers are calculated by the Tukey ‘s method. Three mice per group.