The coordinated secretion of ions, fluid, & mucin into the airway lumen resulting in effective mucus clearance serves to protect the airways from inhaled particulates & pathogens. When the secretion of ions, fluid, or mucin is dysregulated or cilia function is defective, mucus transport & clearance can fail leading to airway infection & disease. CFTR plays a central role in regulating ion & fluid transport in the airways & defective CFTR function is strongly correlated with dysfunctional mucus transport & clearance. This workshop focuses on how ion, fluid, & mucin secretion are regulated in the airways, how regulation of mucus transport is disrupted in CF airways, & which components of the mucus transport process can be targeted therapeutically to restore defective mucus transport.
Identify the contribution of ion, fluid, & mucin secretion to effective mucus clearance in the airways & why it may fail in CF airways.
Differentiate between the mucin species secreted by the proximal & distal airways & their biological significance.
Evaluate the importance of ion, fluid, &/or mucin secretion as therapeutic targets for enhancing mucus clearance and assess the utility of new in vivo techniques for measuring mucus clearance in humans.