The cystic fibrosis neutrophil: a specialized yet potentially defective cell

Arch Immunol Ther Exp (Warsz). 2011 Apr;59(2):97-112. doi: 10.1007/s00005-011-0113-6. Epub 2011 Feb 11.


Cystic fibrosis (CF) is one of the commonest genetically inherited diseases in the world. It is characterized by recurrent respiratory tract infections eventually leading to respiratory failure. One of the hallmarks of this disease is a persistent and predominantly neutrophil driven inflammation. Neutrophils provide the first line of defence by killing and digesting phagocytosed bacteria and fungi, yet despite advances in our understanding of the molecular and cellular basis of CF, there remains a paradox of why recruited CF neutrophils fail to eradicate bacterial infections in the lung. This review describes mechanisms involved in neutrophil migration, microbial killing and apoptosis leading to inflammatory resolution. We discuss dysregulated neutrophil activity and consider genetic versus inflammatory neutrophil reprogramming in CF and ultimately pharmacological modulation of the CF neutrophil for therapeutic intervention.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Apoptosis
  • Cell Degranulation
  • Chemotaxis, Leukocyte
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / immunology*
  • Cystic Fibrosis / metabolism
  • Cystic Fibrosis / microbiology
  • Cystic Fibrosis / pathology
  • Cystic Fibrosis / therapy
  • Humans
  • NADPH Oxidases / metabolism
  • Neutrophil Activation*
  • Neutrophils / immunology*
  • Neutrophils / metabolism
  • Neutrophils / microbiology
  • Neutrophils / pathology
  • Phagocytosis
  • Respiratory Burst


  • NADPH Oxidases