Fig. 6
IMS-088 treatment of hFUSR521G/Syn1 mice restores neuromorphology and synapses. a Neurolucida tracing of cortical motor neuron dendrites from vehicle and IMS-088 treated hFUSR521G/Syn1 mice. b Sholl analysis shows a significant increase in the dendritic intersections and cumulative area of dendrites in cortical motor neurons of hFUSR521G/Syn1 mice treated with IMS-088 (IMS) compared to vehicle (veh) treated mice. c Golgi images of dendritic spines from layer IV–V neurons in the motor cortex from treated mice and d their quantification. There is a significant increase in the dendritic spine density of mature spines of hFUSR521G/Syn1 mice treated with IMS-088. e Neurolucida tracing of the dendrites of spinal motor neurons from hFUSR521G/Syn1 mice treated mice. f Sholl analysis shows some improvement in dendritic intersections of IMS-088 treated mice, but no improvement in cumulative area of dendrites in spinal motor neurons. g The spinal cord of CTL and hFUSR521G/Syn1 mice stained with anti-ChAT (motor neuron marker) and anti-NeuN. h Quantification of spinal motor neuron co-stained with ChAT and NeuN. i Neuromuscular junctions from gastrocnemius stained with α-bungarotoxin-647 (BTX) and anti-synaptophysin (SYP). j Quantification of neuromuscular junctions co-stained with BTX and SYP. Values from each group are expressed as mean ± SEM. Statistics uses two-way repeat measures ANOVA for Sholl analysis and one-way ANOVA for multiple group comparisons (n = 3–4 mice/group). *p < 0.05, **p < 0.01, ***p < 0.005 ****p < 0.001, and not significant (ns). For multiple group comparisons: black (*)/ns: CTL(veh) versus hFUSR521G/Syn1(veh), orange (*)/ns: CTL (veh) versus hFUSR521G/Syn1(IMS) and blue (*)/ns: hFUSR521G/Syn1(veh) versus hFUSR521G/Syn1(IMS)