Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6240360 | Journal of Cystic Fibrosis | 2016 | 7 Pages |
BackgroundIn CF patients, the defective ion transport causes a simultaneous reduction of fluid, Clâ and HCO3â secretion. We aimed to demonstrate that the resulting altered properties of mucus can be recovered using lumacaftor, a CFTR corrector.MethodsThe micro-rheology of non-CF and CF mucus was analysed using Multiple Particle Tracking.ResultsThe 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 HCO3â towards the airway surface fluid, improved CF mucus properties.ConclusionCF mucus properties can be recovered by either improving the hydration of the airways or recovering Clâ and HCO3â secretion across the mutated protein treated with a corrector compound.