Bench-to-bedside review: The role of glycosaminoglycans in respiratory disease |
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Authors: | Alba B Souza-Fernandes Paolo Pelosi Patricia RM Rocco |
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Affiliation: | 1. Laboratory of Pulmonary Investigation, Carolos Chagas Filho Biophysics Institute, Federal University of Rio de Janeiro, Ilha do Fund?o, 21949-900, Rio de Janeiro, Brazil 2. Department of Ambient, Health and Safety, University of Insubria, Viale Borri 57, 21100, Varese, Italy 3. Laboratory of Pulmonary Investigation, Carlos Chagas Filho Biophysics Institute, Federal University of Rio de Janeiro, Ilha do Fund?o, 21949-900, Rio de Janeiro, Brazil
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Abstract: | The extracellular matrix (ECM) plays a significant role in the mechanical behaviour of the lung parenchyma. The ECM is composed of a three-dimensional fibre mesh that is filled with various macromolecules, among which are the glycosaminoglycans (GAGs). GAGs are long, linear and highly charged heterogeneous polysaccharides that are composed of a variable number of repeating disaccharide units. There are two main types of GAGs: nonsulphated GAG (hyaluronic acid) and sulphated GAGs (heparan sulphate and heparin, chondroitin sulphate, dermatan sulphate, and keratan sulphate). With the exception of hyaluronic acid, GAGs are usually covalently attached to a protein core, forming an overall structure that is referred to as proteoglycan. In the lungs, GAGs are distributed in the interstitium, in the sub-epithelial tissue and bronchial walls, and in airway secretions. GAGs have important functions in lung ECM: they regulate hydration and water homeostasis; they maintain structure and function; they modulate the inflammatory response; and they influence tissue repair and remodelling. Given the great diversity of GAG structures and the evidence that GAGs may have a protective effect against injury in various respiratory diseases, an understanding of changes in GAG expression that occur in disease may lead to opportunities to develop innovative and selective therapies in the future. |
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