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The extracellular matrix - the under-recognized element in lung disease?

Burgess, Janette K; Mauad, Thais; Tjin, Gavin; Karlsson, Jenny C LU and Westergren-Thorsson, Gunilla LU (2016) In Journal of Pathology 240(4). p.397-409
Abstract

The lung is composed of airways and lung parenchyma, and the extracellular matrix (ECM) contains the main building blocks of both components. The ECM provides physical support and stability to the lung, and as such it has in the past been regarded as an inert structure. More recent research has provided novel insights revealing that the ECM is also a bioactive environment that orchestrates the cellular responses in its environs. Changes in the ECM in the airway or parenchymal tissues are now recognized in the pathological profiles of many respiratory diseases, including asthma, chronic obstructive pulmonary disease (COPD), and idiopathic pulmonary fibrosis (IPF). Only recently have we begun to investigate whether these ECM changes... (More)

The lung is composed of airways and lung parenchyma, and the extracellular matrix (ECM) contains the main building blocks of both components. The ECM provides physical support and stability to the lung, and as such it has in the past been regarded as an inert structure. More recent research has provided novel insights revealing that the ECM is also a bioactive environment that orchestrates the cellular responses in its environs. Changes in the ECM in the airway or parenchymal tissues are now recognized in the pathological profiles of many respiratory diseases, including asthma, chronic obstructive pulmonary disease (COPD), and idiopathic pulmonary fibrosis (IPF). Only recently have we begun to investigate whether these ECM changes result from the disease process, or whether they constitute a driving factor that orchestrates the pathological outcomes. This review summarizes our current knowledge of the alterations in the ECM in asthma, COPD, and IPF, and the contributions of these alterations to the pathologies. Emerging data suggest that alterations in the composition, folding or rigidity of ECM proteins may alter the functional responses of cells within their environs, and in so doing change the pathological outcomes. These characteristics highlight potential avenues for targeting lung pathologies in the future. This may ultimately contribute to a better understanding of chronic lung diseases, and novel approaches for finding therapeutic solutions. © 2016 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Asthma, Extracellular Matrix, Extracellular Matrix Proteins, Glucocorticoids, Humans, Idiopathic Pulmonary Fibrosis, Lung Diseases, Pulmonary Disease, Chronic Obstructive, Journal Article, Review
in
Journal of Pathology
volume
240
issue
4
pages
13 pages
publisher
John Wiley & Sons
external identifiers
  • scopus:85010743662
  • wos:000387778300004
ISSN
0022-3417
DOI
10.1002/path.4808
language
English
LU publication?
yes
id
63ab5f8a-2957-4f4f-a935-8cf92773f8f1
date added to LUP
2017-06-27 13:44:03
date last changed
2017-10-01 05:36:16
@article{63ab5f8a-2957-4f4f-a935-8cf92773f8f1,
  abstract     = {<p>The lung is composed of airways and lung parenchyma, and the extracellular matrix (ECM) contains the main building blocks of both components. The ECM provides physical support and stability to the lung, and as such it has in the past been regarded as an inert structure. More recent research has provided novel insights revealing that the ECM is also a bioactive environment that orchestrates the cellular responses in its environs. Changes in the ECM in the airway or parenchymal tissues are now recognized in the pathological profiles of many respiratory diseases, including asthma, chronic obstructive pulmonary disease (COPD), and idiopathic pulmonary fibrosis (IPF). Only recently have we begun to investigate whether these ECM changes result from the disease process, or whether they constitute a driving factor that orchestrates the pathological outcomes. This review summarizes our current knowledge of the alterations in the ECM in asthma, COPD, and IPF, and the contributions of these alterations to the pathologies. Emerging data suggest that alterations in the composition, folding or rigidity of ECM proteins may alter the functional responses of cells within their environs, and in so doing change the pathological outcomes. These characteristics highlight potential avenues for targeting lung pathologies in the future. This may ultimately contribute to a better understanding of chronic lung diseases, and novel approaches for finding therapeutic solutions. © 2016 The Authors. The Journal of Pathology published by John Wiley &amp; Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.</p>},
  author       = {Burgess, Janette K and Mauad, Thais and Tjin, Gavin and Karlsson, Jenny C and Westergren-Thorsson, Gunilla},
  issn         = {0022-3417},
  keyword      = {Asthma,Extracellular Matrix,Extracellular Matrix Proteins,Glucocorticoids,Humans,Idiopathic Pulmonary Fibrosis,Lung Diseases,Pulmonary Disease, Chronic Obstructive,Journal Article,Review},
  language     = {eng},
  number       = {4},
  pages        = {397--409},
  publisher    = {John Wiley & Sons},
  series       = {Journal of Pathology},
  title        = {The extracellular matrix - the under-recognized element in lung disease?},
  url          = {http://dx.doi.org/10.1002/path.4808},
  volume       = {240},
  year         = {2016},
}