Fig. 6. Analysis of corrector combinations.
(A and B) F508del-CFTR activity determined in CFBE41o− cells (A) or FRT cells (B) with the HS-YFP. Cells were treated with 4172 (10 μM), ARN23765 (10 nM), or VX-809 (1 μM), as single agents or as combinations. ***P < 0.001 (ANOVA with Tukey’s post hoc test). (C and D) Effect of single agents and combinations in F508del/F508del bronchial epithelial cells with the short-circuit current technique. *P < 0.05, **P < 0.01, and ***P < 0.001 (ANOVA with Tukey’s post hoc test). (E) Evaluation of ARN23765/VX-809 combination. The graphs report F508del-CFTR activity (QR) in CFBE41o− cells treated with VX-809 (1 μM), ARN23765 (10 nM), or both. There was no additive effect elicited by the corrector combination. **P < 0.01 versus vehicle (ANOVA with Dunnett’s post hoc test). (F) Evaluation of F508del-CFTR rescue on apical fluid pH. Experiments were done on F508del/F508del cultured bronchial epithelia treated for 24 hours with ARN23765 (10 nM), 4172 (10 μM), or both compounds together. *P < 0.05, **P < 0.01, and ***P < 0.001 (ANOVA with Tukey’s post hoc test).