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The Cystic Fibrosis Neutrophil: A Specialized Yet Potentially Defective Cell

  • REVIEW
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Archivum Immunologiae et Therapiae Experimentalis Aims and scope

Abstract

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.

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Acknowledgments

We would like to thank the Medical Research Charities Group and the Health Research Board, The Children’s Research Centre, Crumlin Hospital, The US Alpha One Foundation and the Program for Research in Third Level Institutes administered by the Higher Education Authority for support.

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  1. Respiratory Research Division, Department of Medicine, Education and Research Centre, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, Ireland

    Elaine Hayes, Kerstin Pohl, Noel G. McElvaney & Emer P. Reeves

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  1. Elaine Hayes
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  2. Kerstin Pohl
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  3. Noel G. McElvaney
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  4. Emer P. Reeves
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Correspondence to Emer P. Reeves.

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N. G. McElvaney and E. P. Reeves share joint senior authorship.

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Hayes, E., Pohl, K., McElvaney, N.G. et al. The Cystic Fibrosis Neutrophil: A Specialized Yet Potentially Defective Cell. Arch. Immunol. Ther. Exp. 59, 97–112 (2011). https://doi.org/10.1007/s00005-011-0113-6

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