Fig. 6.

Native carboxyl terminal sequences decrease GR dipeptide repeat licited toxicity in rodent neurons. a Top: Schematic of constructs generated to evaluate impact of including an AUG codon in each individual reading frame of the repeat while maintaining the native C-terminal sequence across all 3 reading frames. Bottom: Survival of neurons expressing the indicated constructs as measured by cumulative risk of death. b Schematic of constructs used in panels c-e. c Cumulative risk of death of neurons transfected with constructs containing an AUG codon in the GA reading frame with or without the native C-terminus. d) Cumulative risk of death of neurons transfected with constructs containing an AUG codon in the GP reading frame with or without the native C-terminus. e Cumulative risk of death of neurons transfected with constructs containing an AUG codon in the GR reading frame with or without the native C-terminus. *p < 0.05 and ** p < 0.01 indicate a difference in survival as determined by Cox proportional hazard analysis. The number of neurons for each analysis were as indicated in the respective lines. Each graph represents at least 3 independent experiments