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Burgstaller, G. ; Oehrle, B. ; Gerckens, M. ; White, E.S.* ; Schiller, H. B. ; Eickelberg, O.*

The instructive extracellular matrix of the lung: Basic composition and alterations in chronic lung disease.

Eur. Respir. J. 50:1601805 (2017)
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The pulmonary extracellular matrix (ECM) determines the tissue architecture of the lung, and provides mechanical stability and elastic recoil, which are essential for physiological lung function. Biochemical and biomechanical signals initiated by the ECM direct cellular function and differentiation, and thus play a decisive role in lung development, tissue remodelling processes and maintenance of adult homeostasis. Recent proteomic studies have demonstrated that at least 150 different ECM proteins, glycosaminoglycans and modifying enzymes are expressed in the lung, and these assemble into intricate composite biomaterials. These highly insoluble assemblies of interacting ECM proteins and their glycan modifications can act as a solid phase-binding interface for hundreds of secreted proteins, which creates an information-rich signalling template for cell function and differentiation. Dynamic changes within the ECM that occur upon injury or with ageing are associated with several chronic lung diseases. In this review, we summarise the available data about the structure and function of the pulmonary ECM, and highlight changes that occur in idiopathic pulmonary fibrosis (IPF), pulmonary arterial hypertension (PAH), chronic obstructive pulmonary disease (COPD), asthma and lung cancer. We discuss potential mechanisms of ECM remodelling and modification, which we believe are relevant for future diagnosis and treatment of chronic lung disease.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords Idiopathic Pulmonary-fibrosis; Airway Smooth-muscle; Glycation End-products; Arterial-hypertension; Integrin Adhesome; Animal-models; Proteomic Analysis; Cell-adhesion; Stem-cells; Myosin-ii
ISSN (print) / ISBN 0903-1936
e-ISSN 1399-3003
Journal European Respiratory Journal
Quellenangaben Volume: 50, Issue: 1, Pages: , Article Number: 1601805 Supplement: ,
Publisher European Respiratory Society
Publishing Place Sheffield
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) Institute of Lung Health and Immunity (LHI)
German Center for Lung Research (DZL)