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. 2021 Nov 22;10(11):3260. doi: 10.3390/cells10113260

Figure 1.

Figure 1

Exposure of CF HBE to SMM enhances F508del CFTR rescue. (A–D): CF HBE (F508del/F508del) cultures were exposed for 24 h to vehicle or 5 µM VX-809 in combination with 30 µL mucosal PBS or SMM. (A) Representative short-circuit currents (Isc, µA/cm2) recorded in Ussing chambers. (B) Quantification of F508del-mediated responses to forskolin (Fsk, 10 µM) + VX-770 (1 µM) and CFTRinh-172 (10 µM). (C) Representative Western blot of immunoprecipitated CFTR and tubulin controls. (D) Quantification of band C as % values normalized from band C values from control (vehicle- and PBS-treated CF HBE). (E) SMM overcomes chronic VX-770 treatment (5 μM, 48 h)-mediated abrogation of VX-809-promoted F508del CFTR rescue. (F) SMM overcomes chronic VX-770 treatment (5 μM, 48 h)-mediated abrogation of VX-661-dependent F508del rescue. (G) SMM enhances triple combination-promoted F508del CFTR rescue. CFTR responses to acute forskolin + VX-770 from F508del/F508del HBE in Ussing chambers. HBE were treated with VX-659 (1 µM), VX-661 (5 µM), and VX-770 (5 µM). All data are expressed as mean ± SEM. (A–F): n = 3–4 CF HBE donors, 3–4 cultures/donor. (G): 2 CF HBE donors, 5–6 cultures/donor. * p < 0.05. (A–D): Modified from Gentzsch et al., 2018 [58] (reproduced with permission of the ©ERS 2021: European Respiratory Journal 52 (6) 1801133; doi: 10.1183/13993003.01133-2018 Published 20 December 2018). (E–G): Modified from Gentzsch et al., 2021 [32].