1. Pharmacological rescue of mutant CFTR protein improves the viscoelastic properties of CF mucus 
Ambra Gianotti, Valeria Capurro, Paolo Scudieri, Luis J.V. Galietta, Oscar Moran, Olga Zegarra-Moran 
Journal of Cystic Fibrosis 
Volume 15, Issue 3, Pages (May 2016)
DOI: /j.jcf
Copyright © 2015 European Cystic Fibrosis Society. Terms and Conditions

2. Fig. 1
Morphologic and electrophysiological properties showing a mature airway epithelium. A) Immunofluorescence of 4-week old bronchial epithelium presented in xy and zx views, as indicated. The cilia are labelled in magenta (anti tubulin antibody), the goblet cells in red (anti MUC5AC antibody), the apical membrane in green (anti TMEM16A antibody) and the nucleus in blue (Hoechst dye). B) In vivo fluorescence staining of airway mucus (orange, see Methods). The apical membrane is stained in green and the nucleus in turquoise. C) Short circuit currents (Isc) measured in non-CF, in untreated CF and in lumacaftor treated CF epithelia. Lumacaftor incubation lasted 48h. ENaC was blocked with apical amiloride and CFTR activation and block was obtained by adding CPTcAMP and CFTRinh−172, respectively. D) and E) show the mean±SEM of CFTR-dependent and ENaC-dependent Isc on non-CF (n=23), untreated CF (n=26) and lumacaftor treated CF epithelia (n=12). Asterisks indicate that the difference was statistically significant, p<0.05.
Journal of Cystic Fibrosis  , DOI: ( /j.jcf )
Copyright © 2015 European Cystic Fibrosis Society. Terms and Conditions

3. Fig. 2
Rate of fluid absorption of non-CF and CF epithelia and protein content of released mucus. A) Net fluid absorption measured on non-CF epithelia and on CF epithelia, either untreated or incubated with lumacaftor, lumacaftor plus ivacaftor or with amiloride. B) Protein concentration determined on the ASF recovered from non-CF and CF epithelia. The number of experiments done in each condition is indicated. Bars are SEM, * and ** indicate a p<0.05 or p<< 0.001, respectively.
Journal of Cystic Fibrosis  , DOI: ( /j.jcf )
Copyright © 2015 European Cystic Fibrosis Society. Terms and Conditions

4. Fig. 3
Micro-rheology of epithelial mucus. A) Displacement of representative beads in non-CF and CF mucus. B) Distribution of Do measured on non-CF and CF mucus (n=19 and 26, respectively). The difference was statistically significant (p<0.005). C) Representative experiments displaying 252 and 460 trajectories of beads in non-CF and CF mucus, respectively, together with the mean square displacement and the best fit (dashed and continuous lines, respectively). D) The diffusion coefficient Do measured in the ASF recovered from non-CF epithelia and from CF epithelia, either untreated or incubated 48h with lumacaftor alone, with lumacaftor plus ivacaftor, or with amiloride. The number of experiments done in each condition is indicated. Bars are SEM, * and ** indicate a p<0.05 or a p<0.01, respectively.
Journal of Cystic Fibrosis  , DOI: ( /j.jcf )
Copyright © 2015 European Cystic Fibrosis Society. Terms and Conditions

5. Fig. 4
Frequency-dependent of viscoelastic parameters in epithelial mucus. A) The elastic (G′) and the viscous (G′′) moduli measured in non-CF, CF and lumacaftor- or amiloride-treated CF mucus (n=7). B) The picture displays tan δ (G′′/G′) vs frequency and indicates that the ratio between G′′ and G′ in the three conditions were similar: the higher values of elastic and viscous modules in CF were constant across all frequencies.
Journal of Cystic Fibrosis  , DOI: ( /j.jcf )
Copyright © 2015 European Cystic Fibrosis Society. Terms and Conditions