Figure 1

Potential sites for the effects of CF airway modifiers. Genes that affect the expression of CFTR, processing of the CFTR protein, or activity of ion channels in the epithelium, as well as control of mucus production and mucociliary clearance (left), could all have an impact on the sequence of events in the CF lung. Genes that influence the consequences of the defects at the epithelium, such as infection, chemotactic signals of inflammation, and tissue remodeling, would also be likely to modify clinical manifestations of the disease.