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.