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. 2021 Oct 25;11:21295. doi: 10.1038/s41598-021-00539-5

Figure 4.

Figure 4

Synergism of ivacaftor (VX-770) and elexacaftor (VX-445) in potentiating G551D-CFTR in HNE cells. (A) Representative It recordings of G551D-HNE cells showing acute actions of VX-770 and VX-445. (B–C) Changes in It after acute addition of VX-770 in the absence and presence of VX-445 (B) and in response to the acute addition of VX-445 in the absence and presence of VX-770 (C). (D–E) Changes in It after the additions of test compounds for the experiment presented in (A). G551D-CFTR mediated It is greatest after acute potentiation by both VX-770 and VX-445. (F) Representative It recordings of G551D-HNE treated for 24 h with DMSO, the double combination of VX-770 and VX-445, or the triple combination of VX-661, VX-770, and VX-445 (i.e., Trikafta). (G) CFTRinh-172 inhibited It for the experiment presented in (F). 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 < 0.0001.