Fig. 5

Native carboxyl terminal sequences reduce GGGGCC repeat toxicity in rodent neurons and in flies. a Top: Schematic of the constructs transfected into neurons, with GFP placed in different reading frames relative to the GGGGCC repeat. Bottom: Neuronal survival of neurons expressing these constructs is graphed as the cumulative risk of death (Y axis, with higher values reflecting increased death) over time (X-axis, measured in hours). b Top: Schematic of the constructs transfected into neurons with shorter or expanded G4C2 repeats and with or without the native Cterminus. Bottom: Cumulative risk of death of neurons. c Top: Schematic of the constructs used to generate transgenic fly lines. Bottom: Representative eye phenotypes from flies of the indicated genotypes crossed to GMR-GAL4 to drive expression in developing ommatidia (left) and quantification of eye phenotype scores (right). Graphs represent means ± SEM. For Panels A and B, n = number of neurons. **p < 0.01 difference in survival measured by Cox proportional hazard analysis. Each graph represents at least 3 independent experiments. For panel C, ** p < 0.01 by Kruskal-Wallis after Dunn’s correction for multiple comparisons compared to control group. † p < 0.01 by Kruskal-Wallis after Dunn’s correction for multiple comparisons compared to (G4C2)69 CT group. The number of flies analyzed in panel C was > 100