| Autor: |
Mazio C; Istituto Italiano di Tecnologia (IIT)─Center for Advanced Biomaterials for Healthcare, Largo Barsanti e Matteucci 53, 80125 Napoli, Italy., Scognamiglio LS; Istituto Italiano di Tecnologia (IIT)─Center for Advanced Biomaterials for Healthcare, Largo Barsanti e Matteucci 53, 80125 Napoli, Italy., Passariello R; Istituto Italiano di Tecnologia (IIT)─Center for Advanced Biomaterials for Healthcare, Largo Barsanti e Matteucci 53, 80125 Napoli, Italy.; Department of Chemical, Materials and Industrial Production Engineering (DICMAPI), University of Naples Federico II, P.le Tecchio 80, 80125 Naples, Italy., Panzetta V; Department of Chemical, Materials and Industrial Production Engineering (DICMAPI), University of Naples Federico II, P.le Tecchio 80, 80125 Naples, Italy.; Interdisciplinary Research Centre on Biomaterials (CRIB), University of Napoli Federico II, P.le Tecchio 80, 80125 Napoli, Italy., Casale C; Interdisciplinary Research Centre on Biomaterials (CRIB), University of Napoli Federico II, P.le Tecchio 80, 80125 Napoli, Italy., Urciuolo F; Department of Chemical, Materials and Industrial Production Engineering (DICMAPI), University of Naples Federico II, P.le Tecchio 80, 80125 Naples, Italy.; Interdisciplinary Research Centre on Biomaterials (CRIB), University of Napoli Federico II, P.le Tecchio 80, 80125 Napoli, Italy., Galietta LJV; Telethon Institute of Genetics and Medicine (TIGEM), Via Campi Flegrei 34, 80078 Pozzuoli (NA), Italy., Imparato G; Istituto Italiano di Tecnologia (IIT)─Center for Advanced Biomaterials for Healthcare, Largo Barsanti e Matteucci 53, 80125 Napoli, Italy., Netti PA; Istituto Italiano di Tecnologia (IIT)─Center for Advanced Biomaterials for Healthcare, Largo Barsanti e Matteucci 53, 80125 Napoli, Italy.; Department of Chemical, Materials and Industrial Production Engineering (DICMAPI), University of Naples Federico II, P.le Tecchio 80, 80125 Naples, Italy.; Interdisciplinary Research Centre on Biomaterials (CRIB), University of Napoli Federico II, P.le Tecchio 80, 80125 Napoli, Italy. |
| Abstrakt: |
Cystic fibrosis (CF) is one of the most frequent genetic diseases, caused by dysfunction of the CF transmembrane conductance regulator (CFTR) chloride channel. CF particularly affects the epithelium of the respiratory system. Therapies aim at rescuing CFTR defects in the epithelium, but CF genetic heterogeneity hinders the finding of a single and generally effective treatment. Therefore, in vitro models have been developed to study CF and guide patient therapy. Here, we show a CF model on-chip by coupling the feasibility of the human bronchial epithelium differentiated in vitro at the air-liquid interface and the innovation of microfluidics. We demonstrate that the dynamic flow enhanced cilia distribution and increased mucus quantity, thus promoting tissue differentiation in a short time. The microfluidic devices highlighted differences between CF and non-CF epithelia, as shown by electrophysiological measures, mucus quantity, viscosity, and the analysis of ciliary beat frequency. The described model on-chip may be a handy instrument for studying CF and setting up therapies. As a proof of principle, we administrated the corrector VX-809 on-chip and observed a decrease in mucus thickness and viscosity. |
