The project is based on the use of two lipids, GM1 (Monosialic Ganglioside type 1) and cholesterol to increase the stability of the mutated CFTR channel. Continuation of the previous FFC#2/2020, this project is aimed at evaluating the adjuvant effect of GM1 and cholesterol with Kaftrio/Trikafta on orphan CFTR mutations. Given that the functionality of the plasma membrane, site of the CFTR channel, is impaired in people with cystic fibrosis (CF) with Pseudomonas aeruginosa (PA) infections, the research group will evaluate whether lipids also have positive effects on the infection. The research group wants to study the effect of these lipids on the interaction of PA with the respiratory epithelium and on the activation of the inflammatory response following infection, regardless of the mutations present. Studies in CF cells with different genotypes will be conducted using both cultures of bronchial epithelium cells and primary human respiratory tract cells. The possible effect of lipids on PA infection will also be evaluated in mouse models of CF with chronic PA infection.