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. Author manuscript; available in PMC: 2023 Feb 3.
Published in final edited form as: Eur Respir J. 2022 Feb 3;59(2):2100185. doi: 10.1183/13993003.00185-2021

Figure 4. ETI treatment of F508del cells restores CFTR function and affects mucin concentration but not ASL pH.

Figure 4.

Primary HBE cells homozygous for F508del were treated for 3 days with 0.06% DMSO or 3μM VX-661, 2μM VX-445 and 1μM VX-770 (ETI) before experimentation. A.) Bioelectrical measurements of CFTR-mediated Isc for non-treated (NT) and ETI-treated F508del cells showing cell response to forskolin (Fsk) (i.) and CFTR inhibitor (I-172) (ii.). n=3 per group **P<0.01, Student’s t-test. B.) ASL pH of DMSO and VX-770-treated cells measured by microprobe under CO2 and temperature-controlled conditions. n=6 per group. C.) Absolute mucin concentration in 30 min apical washings as measured via SEC-MALS. n=6 per group. ***P<0.001, Student’s t-test. Error bars indicate SEM.