C9-miR zebrafish displayed TDP-43 pathology.a Illustration of 6 dpf zebrafish skeletal muscle cells with large nuclei labelled with muscle marker, phalloidin, and nucleus marker, Hoechst. b Representative images of 6 dpf zebrafish skeletal muscle cells for TDP-43. Compared to control fish, we observed cytoplasmic clustering of TDP-43 expression in the C9-miR skeletal muscles that can be rescued in C9-rescue fish. Arrows indicate clusters of TDP-43 expression. c Quantification of the nucleus-to-cytoplasmic ratio for TDP-43. A significant reduction in N-to-C for TDP-43 was observed in C9-miR zebrafish (n = 7; **p < 0.005; one-way ANOVA) that can be significantly rescued upon the expression of the human C9orf72 mRNA (n = 5; *p < 0.05; one-way ANOVA). Scale bar = 50 μm. Data are presented as mean ± SEM. n represents the number of fish.
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