The aims of the project were: 1) to investigate whether the bicarbonate treatment modifies the water reabsorption in the ASL;2) to verify the role of the bicarbonate direct treatment on the pH of the ASL; 3) to study the role of the airway bicarbonate on the viscoelastic properties of the ASL mucus to examine whether the mutant CFTR rescue by lumacaftor is correlated with the bicarbonate transport and pH modifications in the ASL. Primary bronchial cells monolayers from normal subjects and CF patients were used as epithelial models. Bicarbonate was directly applied to the apical surface of the monolayers. The results clearly indicate that the treatment with bicarbonate determines an increase of ASL pH and significantly reduces the mucus viscosity in treated CF epithelia. Data obtained in this work will be useful for the design strategies for CF treatment patients using inhaled bicarbonate and improve the protocols that are used in a clinical trial. Noteworthy, bicarbonate might represent a mutation-independent and low-cost therapy to clear out the mucus that accumulates in the airways, reducing the risk of infections, and improve lung function.

Congress abstracts

– Baroni D, Moran O and Ferrera L “Lumacaftor-rescued F508del-CFTR has a reduced bicarbonate permeability” 15th ECFS Basic Science Conference, Loutraki (Greece), March 21-24, 2018
– Ferrera L, Gianotti A, Delpiano L et al. “Bicarbonate directly reduces the mucus micro-viscosity in primary bronchial cells monolayers” 15th ECFS Basic Science Conference, Loutraki (Greece), March 21-24, 2018