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Fig. 5 | Acta Neuropathologica Communications

Fig. 5

From: Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition

Fig. 5

hFUSR521G/Syn1 mice treated with IMS-088 improves cognitive and motor function. a Chemical structure of IMS-088. b Schematic of treatment and behavior analysis of mice. Dotted line indicates when behavior tests were performed. Treatment of hFUSR521G/Syn1 mice with IMS-088 (IMS) improves their cognitive and motor performance compared with vehicle (veh) treated littermate control (CTL) and hFUSR521G/Syn1 mice. hFUSR521G/Syn1 mice treated with IMS-088 display c significant improvement in novel object recognition and d modest improvement in the passive avoidance test. FUS transgenic mice treated with IMS-088 display improved motor function as determined by e hindlimb splay, f wire hanging, and g rotarod tests. Values from each group are expressed as mean ± SEM. Statistics uses an unpaired Student’s t-test for comparison between two groups and a one-way ANOVA for multiple group comparisons (n = 9 hFUSR521G/Syn1(veh), n = 9 hFUSR521G/Syn1(IMS) and n = 12 CTL (veh) 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)

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