(A) Representative tracing from a rabbit in a standardized nasal potential difference (NPD) protocol with sequential administration of amiloride, +low Cl− solution, +ivacaftor (10 μM), + GlyH-101(20μM)/CFTRinh-172 (10 μM). (B) Summary data of studies described above. Ivacaftor perfusion resulted in mean NPD polarization that was significantly greater than low Cl− control (n=4; p≤0.01). (C) Representative Ussing chamber current tracings of RNE. Sequential addition of amiloride, ivacaftor or DMSO control, forskolin, and CFTR inh-172 are shown. (D) Ivacaftor significantly increased CFTR-mediated Cl− transport over vehicle controls (n = 10; p < 0.001).