Modeling the airway surface liquid regulation in human lungs

The research in this dissertation is aimed at understanding the pathogenesis of Cystic Fibrosis, which is a life-threatening disorder that causes severe lung damage and nutritional deficiencies. Patients with cystic fibrosis exhibit deficient mucociliary clearance due to enhanced thickness and adherence of the mucus in their airway. In the past a few decades, significant progress has been made in understanding the cause of the disease as well as ways to treat it. Besides conventional treatments for cystic fibrosis, the addition of adenine triphosphate (ATP) to the airway surface of the lung was recently found to be effective to restore mucociliary clearance. Even as scientists begin to understand the signaling pathways that regulate the airway surface liquid (ASL), there are still many parts of this complex system that remain unclear. The ultimate goal of this work is to put together several important puzzle pieces, and try to understand how proper regulation of the ASL is achieved