1. Repeat‐associated non‐AUG translation in C9orf72‐ALS/FTD is driven by neuronal excitation and stress
AFilter‐trap binding assays show that the ISR proteins are more active in HEK293T cells versus NSC34 and cortical neurons. These basal ISR levels correlate with DPR levels produced in basal conditions. n = 4. BAll stressors increase ISR proteins fluorescent intensity levels in iPSC sMNs. These values are identical to those seen in Fig A, but each protein quantification is split for individual statistical analysis using one‐way ANOVA. Changes in ISR protein levels are shown relative to non‐stressed or DMSO‐only‐treated cells (CTRL) depending on the vehicle compounds were dissolved in. n = 4. ****P < 0.0001, ***P < 0.001, **P < 0.01, *P < 0.05. Error bars represent the SEM. CWestern blot analysis shows changes in ISR protein levels are consistent with filter‐trap binding assay results.
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EMBO Mol Med, Volume: 11, Issue: 2, First published: 07 January 2019, DOI: ( /emmm )