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. 2019 Feb 26;2(2):e201800222. doi: 10.26508/lsa.201800222

Figure 7. ALS-associated FUS mutations confer hypersensitivity to TOP1-induced DNA breakage.

Figure 7.

(A) Clonogenic survival of fibroblasts from an ALS patient expressing FUSR521H and an unaffected sibling control (Control) in the presence of the indicated concentrations of CPT (left) or following irradiation at indicated doses (right). Data are the mean (±SEM) of three independent experiments. (B) Clonogenic survival of HeLa cells expressing wild-type GFP-FUS or the ALS-associated mutants GFP-FUSR521C (GFP-R521C) or GFP-FUSP525L (GFP-P525L) in the presence of the indicated concentrations of CPT. Data are the mean (±SEM) of four independent experiments. Statistically significant differences are indicated (two-factor ANOVA; **P < 0.01, ***P < 0.001).