Primary bronchial epithelial cell culture from people with cystic fibrosis (CF) - a model to study CF lung disease

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Over 7000 people have cystic fibrosis (CF) in the United Kingdom making it the most common life-limiting inherited disease. Death and illness in CF are largely due to progressive lung disease. The disease is characterised by severe inflammation, particularly involving neutrophilic pus cells, and structural airway damage. Neutrophilic inflammation is one of the earliest findings in CF occurring in young children prior to obvious infection. Interleukin(IL)-17, a chemical mediator produced by the body, has attracted great interest recently as a driver of neutrophil-pus cells in a range of inflammatory diseases. In CF there is evidence that concentrations of IL-17 rise in sputum when patients become unwell.We are the first group in Europe to establish the invaluable experimental resource of culturing cells from the lungs of actual people with CF at the time of transplantation. The first studies using our new model of CF lung disease will focus on the role of IL-17. An improved understanding of the inflammatory process may lead to new treatments capable of alleviating the inflammation and lung damage that is such a problem in CF.