Figure 5.

Improvement in neuromuscular junction (NMJ) pathology. The longissimus capitis (LC) and the splenius capitis (SC) muscles from ICV injected and control animals at P12 were immunostained for nerve terminals with antineurofilament/antisynaptophysin (Nerve/Syn) (in green) and motor endplates with α-bungarotoxin (in red). While the untreated SMNΔ7 mice displayed typical severe denervation, E1^MOv11 treatment substantially restored NMJ's pretzel-like structures. (a) Selected high-magnification images of NMJs in splenius muscles of control and SMNΔ7 mice are shown. Arrows indicate occupied endplates in both treated and unaffected animals contrasting the empty endplate seen in untreated controls. (b) Quantification of the number of functional NMJs from LC and SC muscles from healthy unaffected mice, SMNΔ7 noninjected controls and E1^MOv11-treated SMNΔ7 mice (n = 3 per group) where ∗∗∗indicates P ≤ 0.001). (c) Quantification of the percentage of fully and partially innervated NMJs in the LC and SC muscle groups (Unaffected n = 6; SMA noninjected n = 6; E1^MOv11-treated n = 6). ICV, intracerebroventricular; SMA, spinal muscular atrophy; SMN, survival motor neuron.