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Fig. 8 | Acta Neuropathologica Communications

Fig. 8

From: Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features

Fig. 8

Heat shock stress response activation is induced by C9orf72 expansions. (a) In lysates from both HEK 293 T and N2A cell lines, DsRed levels are higher in cells transfected with 39 C4G2 pure repeats (Left and middle panel) or 89 C4G2 interrupted repeats (Right panel) compared with those transfected with only 2 C4G2 repeats. (b) At 5dpf, 2.2–7 zebrafish show significantly higher DsRed fluorescence than 2.2–2 zebrafish. N = 75 2.2–2 and 76 2.2–7 individual zebrafish. (c) At 5dpf, GFP fluorescence is not significantly different between 2.2–7 and 2.2–2 zebrafish. N = 75 2.2–2 and 76 2.2–7 individual zebrafish. (d) In end-stage 2.2–7 zebrafish brains, levels of GFP tagged DPR and DsRed proteins are increased compared with pre-symptomatic 2.2–7 and NTG. (e) Quantification of GFP tagged DPR protein normalised to tubulin in adult zebrafish brains. N = 3 adult brains per condition. (f) Quantification of DsRed protein normalised to tubulin in adult zebrafish brains. N = 3 adult brains per condition. (g) In human cerebellum samples, HSP70 protein levels are higher in C9-ALS patients as compared to non-neurological-disease controls. N = 5 samples per group. (h) Quantification of HSP70 protein levels normalised to tubulin in human cerebellum. All data are shown as mean +/− standard deviation; *P < 0.05, **P < 0.01, ***P < 0.001 and ****P < 0.0001

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