The efficacy of Orkambi (Lumacaftor-Ivacaftor) is reduced if Pseudomonas aeruginosa infection is concomitant, so the research group aims to study the effectiveness of old and new modulators in primary human nasal epithelial (HNE) cells in the presence of clinical strains of P. aeruginosa isolated from the corresponding persons with cystic fibrosis (CF). Further objectives of the project are related to the action of cytokines which has been shown to have a role in the pathophysiology of cystic fibrosis. In fact, the research group aims to determine the amount of cytokines released, such as IL-17A, which is a protein secreted by the immune system that has been identified in the sputum of people with CF, to evaluate the levels of gene expression and to study the intracellular signalling pathway. The study will be conducted on HNEs of 15 persons with CF with F508del and other rare mutations infected with a clinical P. aeruginosa strains isolated from the same persons with CF. Project results could provide useful information to optimize the prediction of clinical responses to CFTR modulators.

XIX Convention FFC Ricerca – download here a brief presentation of the project