The cystic fibrosis neutrophil: a specialized yet potentially defective cell.
Affiliation
Respiratory Research Division, Department of Medicine, Education and Research, Centre, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9,, Ireland.Issue Date
2012-02-01T10:00:51ZMeSH
AnimalsApoptosis
Cell Degranulation
Chemotaxis, Leukocyte
Cystic Fibrosis/genetics/*immunology/metabolism/microbiology/pathology/therapy
Humans
NADPH Oxidase/metabolism
*Neutrophil Activation
Neutrophils/*immunology/metabolism/microbiology/pathology
Phagocytosis
Respiratory Burst
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Arch Immunol Ther Exp (Warsz). 2011 Apr;59(2):97-112. Epub 2011 Feb 11.Journal
Archivum immunologiae et therapiae experimentalisDOI
10.1007/s00005-011-0113-6PubMed ID
21311988Abstract
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.Language
engISSN
1661-4917 (Electronic)0004-069X (Linking)
ae974a485f413a2113503eed53cd6c53
10.1007/s00005-011-0113-6
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