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. 2021 Oct 6;11:19810. doi: 10.1038/s41598-021-99184-1

Figure 2.

Figure 2

Effect of chronic treatment with elexacaftor (VX-445) includes its actions as both a CFTR corrector and potentiator. (A) Representative It recordings of non-CF HNE treated for 24 h with DMSO or VX-445 (3 µM). (B–C) Changes in It after the additions of test compounds for the experiment presented in Panel A. The potentiating action of VX-445 accounts for the differences in post-amiloride It between DMSO and VX-445 treated cultures. (D) Representative It recordings of F508del-HNE treated for 24 h with tezacaftor (VX-661; 3 µM), ivacaftor (VX-770; 100 nM), and/or VX-445 (3 µM chronic; 100 nM acute). (E–F) Changes in It after the additions of test compounds for the experiment presented in (D). The triple combination of VX-661/77/445 significantly increased CFTR-mediated It. Acute action of VX-445 in stimulating It was only observed in VX-661/770 treated F508del/F508del nasal cells. See SI for additional experimental details and for supporting data. All data are presented as mean ± standard error. Bars with different letters (A, B, C…) are significantly different from each other (ANOVA; P < 0.05). Asterisks indicate specific P values: ***P < 0.001.