Fig. 5

Induction of PTC readthrough by G418 and enhancers in R493X^−/− KI hiPSC-derived astrocytes. DIV 60+ WT and R493X^−/− KI hiPSC-derived astrocytes were treated with G418, CDX5–288, and rec. Human PGRN at the indicated concentrations for 72 h. Intracellular (a-b, 1 mg/mL lysate) and extracellular (c-d, 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). Therefore, the antibody 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