|Autori||Gianotti A, Capurro V, Scudieri P, Galietta LJ, Moran O, Zegarra-Moran O|
|Abstract||Background: In CF patients, the defective ion transport causes a simultaneous reduction of fluid, Cl- and HCO3- secretion. We aimed to demonstrate that the resulting altered properties of mucus can be recovered using lumacaftor, a CFTR corrector. |
Methods: The micro-rheology of non-CF and CF mucus was analysed using Multiple Particle Tracking.
Results: The diffusion coefficient of nano-beads imbedded in mucus from CF human bronchial epithelium was lower than in non-CF mucus, and the elastic and viscous moduli were higher. We found that 25% correction of F508del-CFTR mutation with lumacaftor was enough to improve significantly CF mucus properties. Surprisingly, also incubation with amiloride, a compound that reduces fluid absorption but might not change the secretion of HCO3- towards the airway surface fluid, improved CF mucus properties.
Conclusion: CF mucus properties can be recovered by either improving the hydration of the airways or recovering Cl- and HCO3- secretion across the mutated protein treated with a corrector compound
|Rivista||Journal Of Cystic Fibrosis|
|Autori IBF||Oscar MORAN|
|Linee di Ricerca IBF||MD.P01.009.001|