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. 2022 Mar 22;7(6):e148841. doi: 10.1172/jci.insight.148841

Figure 5. Single-channel behavior of F312del-CFTR in response to ivacaftor (VX-770).

Figure 5

(A) Representative recordings of an individual F312del-CFTR Cl– channel in an excised inside-out membrane patch from a transiently transfected CHO cell in the absence and presence of the indicated concentrations of ivacaftor (VX-770) in the intracellular solution. Dotted lines indicate where channels are closed and downward deflections correspond to channel openings. (B and C) Summary data show the effects of ivacaftor (10 nM–10 μM) on i and Po of F312del-CFTR. Symbols represent individual values with mean ± SEM indicated (control, n = 8; 10 nM, n = 5; 100 nM, n = 8; 1 μM, n = 6; 10 μM, n = 3). There was no significant difference in the ivacaftor response calculated by 1-way ANOVA (B, P = 0.564; C, P = 0.494). Using similar experimental conditions, we previously demonstrated that ivacaftor (10 nM–10 μM) potentiates WT human CFTR (59).