Cystic Fibrosis (CF) lung disease is characterized by chronic bacterial infection and excessive inflammation. The airways of patients with CF contain large amounts of neutrophils, neutrophil products, and pro-inflammatory mediators. This inflammatory response may be linked to the loss of CFTR function. It is unknown, however, what signaling mechanisms link a loss of CFTR function to the excessive inflammatory response. Several signaling pathways are dysregulated in CF epithelial cells. Among these is the pathway that leads to the production of nitric oxide (NO). Reduced production of NO, which has important antibacterial and anti-inflammatory effects in the airway, may contribute to the establishment of the chronic bacterial infection and the development of the subsequent overzealous inflammatory response in CF.