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. 2019 Sep 17;9:13460. doi: 10.1038/s41598-019-49921-4

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© The Author(s) 2019

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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VX-770 potentiated F508S- and F508C-CFTR similarly to F508del-CFTR. VX-770 (0.2 µM) potentiated F508S-CFTR in high (A) and low (B) PKA concentrations with 1 mM MgATP. Representative current traces of F508C-CFTR potentiated by VX-770 in high (C) and low PKA (D) conditions also are shown. ▼, 10 µM CFTR_inh172. Summary data for F508S- and F508C-CFTR potentiated by VX-770 are shown in (H). F508C: n = 7 for high PKA; n = 6 for low PKA. F508S: n = 6 for high PKA; n = 4 for low PKA. Representative current traces of F508del- (E), F508S- (F), and F508C- (G) CFTR activated by low PKA then by high PKA in the presence of 1 mM MgATP are shown. Summary data for the ratio of three F508 mutants in high and low PKA conditions are shown in panel I and calculated based on the equation in Fig. 1. n = 6 for F508del; n = 6 for F508S; n = 4 for F508C. *P < 0.05 compared to WT.