Figure 9.

CFTR functionality in FRT cells measured by the yellow fluorescent protein (YFP) assay (pink: cells, green: YFP protein expression, blue: multiwell), (A) in YFP-CFTR^WT FRT, and (B) CFTR^G542X and CFTR^W1282X FRT cells, untreated or treated with 12 μM PTC124, NV848, NV914, or NV930 for 24 h (C). Cells were prestimulated with 20 μM forskolin for 20 min. The experiment was performed in triplicate. Images were taken before (t⁰) and after (t^30s) the addition of NaI. Percentages of YFP-positive cells before (− dark green bars) and after NaI addition (+ light green bars); data were analyzed by GraphPad (n = 3) and expressed as mean values ± standard error of the mean (SEM) (D). Data were analyzed comparing treated FRT cells versus untreated FRT cells by GraphPad Prism 6 software (n = 3). ***, p < 0.001 (ANOVA with Dunnett’s posthoc test).