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Volume 11 (2011), Pages 959-971
Mini-Review Article

The Involvement of Glycosaminoglycans in Airway Disease Associated with Cystic Fibrosis

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

Received 6 December 2010; Revised 3 March 2011; Accepted 10 March 2011

Academic Editor: Charles N. Serhan

Copyright © 2011 Emer P. Reeves et al.


Individuals with cystic fibrosis (CF) present with severe airway destruction and extensive bronchiectasis. It has been assumed that these structural airway changes have occurred secondary to infection and inflammation, but recent studies suggest that glycosaminoglycan (GAG) remodelling may be an important independent parallel process. Evidence is accumulating that not only the concentration, but also sulphation of GAGs is markedly increased in CF bronchial cells and tissues. Increased expression of GAGs and, in particular, heparan sulphate, has been linked to a sustained inflammatory response and neutrophil recruitment to the CF airways. This present review discusses the biological role of GAGs in the lung, as well as their involvement in CF respiratory disease, and their potential as therapeutic targets.