Cystic Fibrosis (CF) is the most common genetically determined, early onset and still lethal disease. CF affects approximately one in 2,500 newborns in Germany. With improvements in symptomatic therapies and standardized CF medical care, the median survival age of CF patients in Germany has risen to approximately 40 years. However, despite recent breakthroughs in disease-modifying therapies for a small subgroup of patients with specific CF geno types, there are currently no therapies available to the majority of patients that target CF lung disease at its root. The overall aim of the DZL CF research program is to advance the current understanding of the pathogenesis of CF lung disease and to use this knowledge to improve CF diagnostics, to develop more sensitive tools for monitoring of disease activity, and novel strategies for the effective prevention and therapy of CF lung disease.