Figure 7.

Expression of A234D-CFTR in FRT cells

(A) Ussing chamber assay of FRT cells incubated for 24 h with VX445, VX661 (both at 3 μM), and their combination as indicated. Cells were then treated with amiloride 100 μM (A) followed by forskolin 10 μM (F10, both apical and basolateral side). VX770 (I) 5 μM and CFTRInh 172 (In) 10 μM, we next added to the apical side only in sequence.

(B) Summary of the ΔIsc of three independent experiments. ∗∗p < 0.009 and ∗∗∗∗p < 0.0001 (Ordinary one-way ANOVA).

(C) Representative western blotting image performed using A234D and W57G expressing FRT cells.