Pseudomonas aeruginosa: can studies in engineered cells tell us why is it such a problem in people with cystic fibrosis? Focus on "Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa".

PEOPLE WITH CYSTIC FIBROSIS (CF) are surviving in larger numbers into adulthood and even middle age. Over time, acquisition of antibiotic-resistant gram-positive and gram-negative bacteria in the airways leads to progressive inflammation and cycles of pulmonary exacerbations that drive obstructive lung disease and decline in lung function. CF patients have an exuberant immune system aided by ever more powerful antibiotics that prevent sepsis and death by limiting spread beyond the airways. Yet at the same time, despite aggressive intervention to improve mucociliary clearance and fight infection from infancy, the airways become chronically colonized with specific bacteria that are difficult to eradicate. Periodically, bacteria become more invasive causing pulmonary exacerbations that lead to progressive airways damage and irreversible structural changes. The paradox between a strong immune reaction and failure to eradicate infection supports the hypothesis that there is a primary defect in innate immunity in CF.