title: Targeting neutrophil elastase in cystic fibrosis.
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.
authors.affiliation: Beaumont Hospital, Department of Respiratory Research, RCSI Smurfit Building, Beaumont, Dublin 9, Ireland. emerkelly@rcsi.ie
authors.forename: Emer
authors.initials: E
authors.lastname: Kelly
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Greene
authors.forename: Noel G
authors.initials: NG
authors.lastname: McElvaney
mesh_terms: D003550:Cystic Fibrosis / Q000188:drug therapy*; D004791:Enzyme Inhibitors / Q000494:pharmacology* / Q000627:therapeutic use; D006801:Humans; D019272:Leukocyte Elastase / Q000037:antagonists & inhibitors* / Q000378:metabolism
chemical_list: D004791:Enzyme Inhibitors; D019272:Leukocyte Elastase