MicroRNA Dysregulation in Cystic Fibrosis

Figure 1

The CF airway lumen. Altered ion homeostasis in the CF airway due to mutated CFTR leads to impaired mucociliary clearance and a depleted ASL volume. This, coupled with intrinsic inflammation, leads to chronic bacterial infection and inflammation, with large numbers of neutrophils along with their secreted protease products being recruited to the lung. The high protease burden in the CF airway is damaging to lung tissue and leads to bronchiectasis and ultimately lung failure and death. IL-8: interleukin 8; ASL: airway surface liquid; TLRs: Toll-like receptors; NF-κB: nuclear factor-κB.