Figure 6. Treatment with the p38α MAPK Inhibitor MW150 Prevents Degeneration of SMA Motor Neurons.

(A) ChAT immunostaining of P11 L1 spinal cords from uninjected WT mice and SMA mice treated daily with either saline or MW150 (5 mg/kg). Scale bar: 50 μm.
(B) Total number of L1 motor neurons in the same groups as in (A) at P11. Data represent means and SEM from WT (n = 6), SMA+Vehicle (n = 5), and SMA+MW150 (n = 3). Statistics were performed with one-way ANOVA with Tukey’s post hoc test. **p < 0.01, ***p < 0.001.
(C) ChAT immunostaining of P11 L5 spinal cords from the same groups as in (A). L5 MMC motor neurons are indicated by dotted circles and magnified in the insets. Scale bar: 125 μm.
(D) Total number of L5 MMC motor neurons in the same groups as in (A) at P11. Data represent means and SEM from WT (n = 6), SMA+Vehicle (n = 6), and SMA+MW150 (n = 5). Statistics were performed with one-way ANOVA with Tukey’s post hoc test. **p < 0.01, ***p < 0.001.
(E) Immunostaining of VGluT1+ synapses (gray) and ChAT+ motor neurons (blue) in P11 L1 spinal cords from uninjected WT mice and SMA mice treated daily with either saline or MW150 (5 mg/kg). Scale bar: 20 μm.
(F) Number of VGluT1+ synapses on the somata of L1 motor neurons from the same groups as in (E) at P11. Data represent means and SEM from WT (n = 39), SMA+Vehicle (n = 39), and SMA+MW150 (n = 36). Statistics were performed with one-way ANOVA with Tukey’s post hoc test. ***p < 0.001; ns, not significant.
(G) NMJ staining with bungarotoxin (BTX, red), Synaptophysin (Syp, green), and Neurofilament M (NF-M, blue) of P11 QL muscles from uninjected WT mice and SMA mice treated daily with either saline or MW150 (5 mg/kg). Scale bar: 25 μm.
(H) Percentage of fully denervated NMJs in the QL muscle from the same groups as in (G) at P11. Data represent means and SEM (n = 3). Statistics were performed with one-way ANOVA with Tukey’s post hoc test. **p < 0.01; ns, not significant.