Targeting neutrophil elastase in cystic fibrosis.

BACKGROUND Cystic fibrosis (CF) is a lethal hereditary disease characterised by neutrophil-dominated lung inflammation. These abundant neutrophils produce neutrophil elastase (NE), a destructive serine protease that has direct actions on extracellular matrix proteins and has a role in the host response to inflammation and infection. OBJECTIVE This review examines the prospect of developing novel therapies for CF by targeting NE. The authors explore the functions of NE and of naturally-occurring and synthetic NE inhibitors. METHODS A literature search was conducted exploring the functions of NE and inhibitors of NE; naturally occurring and synthetic. CONCLUSIONS Targeting NE in CF offers therapeutic potential, but optimal inhibitors that can be delivered safely and effectively to the lung are still under development.