Figure 7.
Ivacaftor further stimulates cAMP-dependent CFTR function in CFBE41o− monolayers after translational readthrough. (A and B) CFBE41o− cells stably expressing CFTR-R1162X (A) or W1282X (B) were incubated with synthetic aminoglycosides (250 μg/ml) for 48 hours, followed by Isc measurements in Ussing chambers. Cells were sequentially stimulated with forskolin (100 nM) then ivacaftor (VX-770; 10 μM). Comparisons of total stimulated current, ivacaftor-stimulated current, and forskolin-stimulated current are each designated as compared with control (asterisk noted top to bottom, respectively). The data shown are means ± SEM of at least six replicates per condition. *P < 0.05, **P < 0.01, ***P < 0.001.