Figure 3.
Rescue in CFTR function of rare CFTR mutants by VX-809 and/or VX-770. (A–C) HEK293T cells stably overexpressing untagged CFTR variants were treated with 2.5 µM VX-809 for 24 h (A), 3 µM VX-770 for 20 min (B) or a combination of both (C) prior to determining CFTR function by HS-YFP quenching after stimulation with 10 µM forskolin (Fsk). Graphs depict the mean ± S.D. of three independent experiments represented as dots (DMSO) or squares (compounds). Response to treatment over DMSO was compared for each mutant by multiple t-testing. * P < 0.05, ** P < 0.01, *** P < 0.001, **** P < 0.0001.
