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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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G551D-CFTR current exhibits decreased rate of deactivation upon removal of ATP and PKA. Representative current traces for G551D- (A) and G551A-CFTR (B) activated with 1 mM MgATP + 127.6 U/ml PKA then deactivated by washout of ATP and PKA with intracellular solution (control). A voltage ramp protocol described in the Methods section was applied every 5 s. Summary data for deactivation are shown in (C,D) and follow the equation (I/I_max =I _{(remaining current)}/I _{(maximum current in ATP+PKA)}). ***, P < 0.001 compared with G551A. n = 5 for both G551A and G551D. (E) VX-770 significantly slowed the rate of deactivation of G551D-CFTR upon removal of ATP + PKA + VX-770. Representative current traces for G551D-CFTR activated with 1 mM MgATP + 127.6 U/ml PKA + 0.2 µM VX-770 for about 20 minutes then deactivated by washout with intracellular solution (control) for >25 minutes followed with 10 µM CFTR_inh172 (▼). Summary data for deactivation are shown in (F,G). *P < 0.05 and ***P < 0.001 compared with G551D in the absence of VX-770. n = 5. (H) Representative single channel current traces of WT- and G551D-CFTR recorded in symmetrical 150 mM Cl⁻ solution. Data for G551D-CFTR is from the same patch for pre- and post-VX-770. Summary data for mean burst durations are shown in (I). Mean burst duration for G551D-CFTR was significantly shorter than for WT-CFTR. Mean burst duration for G551D-CFTR in the presence of VX-770 was significantly longer than for ATP + PKA alone. n = 5 for G551D-CFTR. WT-CFTR data cited from previous publication¹⁴. **P < 0.01 compared with WT-CFTR. ***P < 0.001 compared with G551D-CFTR in pre-VX-770 condition.