CF is typically associated with mucus hypersecretion in the airways. In health, mucin is the major macromolecular component and is responsible for the protective and clearance properties of the mucus gel. In a recent study we found that mucins are decreased in the sputum of adult CF patients.

In this study we want to investigate the differences on the mucin and DNA quantity and quality of airway secretions in during pulmoanry exacerbation.We hypothesize that during an exacerbation the mucin and DNA amount is increasing. The aim of this study is to evaluate the molecular (mucins) and structure properties (mucin-DNA-network) of the airway secretions in CF related to the severity of the disease. We characterize sputum composition of patients with pulmonary exacerbations. Using gel electrophoresis and dot-blot with specific antibodies we will analyze MUC5AC and MUC5B mucins. DNA amount will be measured by microfluorimetry.

With the laser scanning confocal microscopy the mucin-DNA-network will be evaluated.

The significance of these studies is that they will give us novel information about the pathogenesis of chronic inflammatory airway diseases, provide tools for assessing the progression of lung disease, and most critically, will identify novel opportunities and targets for therapeutic intervention.