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

被引:192
作者
Burgess, Janette K. [1 ,2 ,3 ,4 ]
Mauad, Thais [5 ]
Tjin, Gavin [2 ,4 ]
Karlsson, Jenny C. [6 ]
Westergren-Thorsson, Gunilla [6 ]
机构
[1] Univ Groningen, Univ Med Ctr Groningen, GRIAC Res Inst, Dept Pathol & Med Biol, Groningen, Netherlands
[2] Univ Sydney, Woolcock Inst Med Res, Resp Cellular & Mol Biol Grp, Glebe, NSW, Australia
[3] Univ Sydney, Discipline Pharmacol, Glebe, NSW, Australia
[4] Univ Sydney, Cent Clin Sch, Glebe, NSW, Australia
[5] Univ Sao Paulo, Sch Med, Dept Pathol, Sao Paulo, Brazil
[6] Lund Univ, Fac Med, Dept Expt Med Sci, Lung Biol, Lund, Sweden
基金
英国医学研究理事会;
关键词
extracellular matrix; lung; collagen; myofibroblast; asthma; chronic obstructive pulmonary disease; niche; fibrosis; AIRWAY SMOOTH-MUSCLE; RETICULAR BASEMENT-MEMBRANE; CONNECTIVE-TISSUE; ELASTIC FIBERS; CELL-PROLIFERATION; TGF-BETA; PROTEOGLYCAN DEPOSITION; BRONCHOALVEOLAR LAVAGE; THERAPEUTIC TARGETS; GENE-EXPRESSION;
D O I
10.1002/path.4808
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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. (c) 2016 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.
引用
收藏
页码:397 / 409
页数:13
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