Fig. 1

Zebrafish axonopathy induced by C9orf72 sense and antisense repeats. a, b Visualization of motor axons by immunohistochemistry (SV2 antibody) of 30 hpf zebrafish embryos injected with 3S (a) or ~ 70S (b) repeat RNA. c, d Axonal length (c) and aberrant axonal branching (d) of zebrafish embryos injected with increasing doses of 3 and ~ 70 sense repeat RNA, compared to GFP control RNA, n = 5 experiments. c Data represent mean ± 95% confidence interval (CI) one-way ANOVA, F(6, 754) = 18.87, ****p < 0.0001. d Data represent mean ± 95% CI logistic regression (z values compared to GFP ? 0.796, ? 0.877, ? 0.200, 3.025, 0.104, 5.703; z values for 3S vs 70S: 1.373, 2.480, 4.538), *p < 0.05, **p < 0.01, ***p < 0.001. e, f Axonal length (e) and aberrant axonal branching (f) of zebrafish embryos injected with sense repeat RNA with a length of 3 (n = 4 experiments), 4 (n = 4 experiments), 10 (n = 4 experiments), ~ 35 (n = 4 experiments), ~ 70 (n = 24 experiments) and ~ 90 (n = 4 experiments) repeats at equimolar dose (0.844 µM). e Data represent mean ± 95% CI one-way ANOVA, F(6, 1140) = 57.16, ****p < 0.0001. f Data represent mean ± 95% CI logistic regression (z values compared to GFP 1.130, ? 0.573, 1.130, 3.035, 3.063, 2.565), *p < 0.05, **p < 0.01. g, h Axonal length (g) and axonal branching (h) of zebrafish embryos injected with antisense repeat RNA with a length of ~ 35 (n = 4 experiments) and ~ 70 (n = 12 experiments) repeats at equimolar dose (0.844 µM). g Data represent mean ± 95% CI one-way ANOVA, F(2, 474) = 35.66, ****p < 0.0001. h Data represent mean ± 95% CI logistic regression (z values compared to GFP 2.450, 3.576), *p < 0.05, **p < 0.01. i?k Whole-mount staining (SV2 antibody) of 30 hpf zebrafish embryos injected with fluorescently labeled 3S, ~ 90S and ~ 70AS repeat RNA