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- 2019
C9orf72 deficiency promotes motor deficits of a C9ALS/FTD mouse model in a dose-dependent mannerDOI: 10.1186/s40478-019-0685-7 Keywords: C9orf72, Motor deficits, C9ALS/FTD, Mice Abstract: C9orf72 dose is critical for motor deficits in C9ALS/FTD mouse models. a Western blot analysis of C9orf72 and Atg101 protein levels in 2-month-old mouse cortex. β-Actin serves as the loading control. b, c Accelerating rotarod test was performed on 4-month-old mice to examine the latency to fall of females (b) and males (c). C9orf72 deficiency decreases the latency to fall of C9-BAC female mice in a dose-dependent manner. d A 4-consecutive-day rotarod assay reveals defective motor learning in C9orf72+/?;C9-BAC and C9orf72?/?;C9-BAC female mice in comparison to WT. e, f Grip strength test was performed to measure front paw strength in 4-month-old females (e) and males (f). All data are presented as mean?±?SEM using numbers (n) of mice as indicated. Statistical analyses were performed with one-way ANOVA with Bonferroni’s post hoc test (*p?<?0.05, **p?<?0.01, ***p?<?0.001, N.s represents no significant difference detected in measurement of 2d, 3d, or 4d in comparison to that of 1d
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