Targeting neutrophil elastase in cystic fibrosis

Expert Opin Ther Targets. 2008 Feb;12(2):145-57. doi: 10.1517/14728222.12.2.145.

Abstract

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.

Publication types

  • Review

MeSH terms

  • Cystic Fibrosis / drug therapy*
  • Enzyme Inhibitors / pharmacology*
  • Enzyme Inhibitors / therapeutic use
  • Humans
  • Leukocyte Elastase / antagonists & inhibitors*
  • Leukocyte Elastase / metabolism

Substances

  • Enzyme Inhibitors
  • Leukocyte Elastase