Fig. 2

Efficiency of double strand DNA break repair pathways in response to dipeptide repeat proteins. a-d) Relative repair efficiency (Y-axis) as determined by the percentage of GFP positive cells in cultures transfected with DPR expression plasmids or an empty vector (set to 100% efficiency). Four reporter cell lines were used to assess the efficiency of (a) homologous recombination (HR), (b) non-homologous end joining (NHEJ), (c) microhomology mediated end joining (MMEJ), and (d) single strand annealing (SSA). Statistical significance was assessed by one-way ANOVA and post-hoc test between each experimental group and the control group (vector); n = 6 biological replicates, 100,000 cells/replicate were evaluated; error bars are SEM, *p < 0.05, ***p < 0.0005, ****p < 0.0001. Inset numbers are the mean difference between groups. E-H) Representative fluorescence activated cell sorting plots of transfected NHEJ and SSA reporter cells using GFP fluorescence (Y-axis) and side scatter (X-axis); the number of GFP positive cells is represented as a percentage of parent gating