Figure 5.

Media selection does not alter F508del CFTR rescue by VX-809; however, it does impact normalization of modulated CFTR function against wtCFTR. Cells grown in four media and treated for 72 h with vehicle or VX-809 were mounted in Ussing chambers and CFTR function was quantified under voltage clamp conditions. Representative tracings of corrected (dashed) and uncorrected (solid) F508del CFTR are shown in (A), while the VX-809-induced improvement in CFTR function is quantified in (B) as a relative change compared to vehicle. When normalized against media-specific wtCFTR function, there is a significant apparent difference in baseline (light grey) and VX-809-modulated (dark grey) F508del CFTR function (C); CFTR function is defined as cAMP- and genistein-dependent Isc, as presented in Figure 4B,D. Baseline resistance measurements for all conditions are shown in (D), with only VX-809-treated F508del inserts grown in ALI media being statistically different across the cohort. n = 3–4 inserts per condition. * p < 0.05; ** p < 0.01; NS: Not Significant vs. MEM by one-way ANOVA with Dunnett’s multiple comparison test (B,C), or two-way ANOVA with Tukey’s multiple comparisons test (D).