This book describes a novel cell biological basis contributing to aberrant innate immunity and inflammation in the cystic fibrosis (CF) respiratory tract. The first aspect covered involves the basis for previously described hyperacidification of organelles in CF cells, and how this phenomenon can be pharmacologically corrected. The second aspect focuses on the excessive production of a molecule named Sialyl-Lewis X (Slx) in CF cells. Slx acts as a receptor for neutrophils which cause excessive damage in the CF bronchial epithelia. The third aspect involves the discovery of attenuated polymeric IgA transcytosis in CF cells, which may contribute to excessive colonization of P. aeruginosa. Future directions addressing these aspects of the cell biology of CF are outlined.