Stem cell transplantation in a cystic fibrosis mouse model induced Cftr genotype conversion and improved outcome of P. aeruginosa lung infection

Infections with Pseudomonas aeruginosa still cause severe morbidity in people with Cystic fibrosis (CF). The Gram-negative, opportunistic pathogen is nearly perfectly adapted to the microaerophilic conditions within the CF lung characterized by impaired ciliary function, mucus plugging and progressive remodeling. This scenario of chronic inflammation and loss of lung function is not only caused by the loss of function of the CFTR channel in the respiratory epithelium, but also by an impaired function of professional phagocytes in CF lung disease as it was demonstrated by several studies during the last years. He…