Fig. 3

Induction of PTC readthrough by G418 and enhancers in hiPSC-derived cortical neurons bearing FTD-GRN nonsense mutations. DIV 50 S116X^+/−, R418X^+/−, R493X^−/− KI, and WT hiPSC-derived cortical neurons were treated with G418 and CDX5–288 at the indicated concentrations for 72 h. Intracellular (a-d, 1 mg/mL lysate) and extracellular (e-h, 25X concentrated supernatant) samples were subjected to PGRN ELISA using a polyclonal anti-PGRN antibody that targets multiple epitopes (GRN 1/3/5/7 peptides) and cannot differentiate between truncated/full-length PGRN or GRN peptides (PGRN/GRNs). n = 3 independent cultures; values are shown as mean ± SEM; * p < 0.05, ** p < 0.01, *** p < 0.0001 was determined by one-way ANOVA with Tukey’s multiple comparison test