Genetic inhibition of ASM prevents motor neuronal loss in the spinal cord of FUS-R521C mice. (A) Left, representative immunofluorescence images of motor neuron (ChAT, green) in the spinal cord of wild type (WT), FUS-R521C, and FUS-R521C; Smpd1+/− mice. Scale bars, 50 μm. Right, quantification of motor neuron survival in each group (n = 4-5 per group). (B) Representative immunofluorescence images of FUS and FUS-R521C protein detected by FUS antibody (blue) and hFUS (Flag) antibody (red). Scale bars, 20 μm. One-way analysis of variance, Tukey’s post hoc test. **P < 0.01, ***P < 0.001. All error bars indicate S.E.M. All data analysis was done on 12-week-old mice.