Fig. 4.
Chronic treatment with ivacaftor and C18 in F508del/F508del HBE decreases CFTR function. A: representative tracings of F508del/F508del HBE monolayers treated with vehicle, C18, or C18 + VX-770. B: change in Isc responses to forskolin stimulation. C: change in Isc in response to acute application of VX-770. D: cumulative change in Isc response to both agonists. E: change in Isc after addition of CFTRInh-172. F: change in sweat chloride concentration in subjects treated with VX-809 or VX-809 and VX-770. n = 4–6. *P < 0.05, **P < 0.01, ***P < 0.001, and ****P < 0.0001.