RT Journal Article
SR Electronic
T1 The instructive extracellular matrix of the lung: basic composition and alterations in chronic lung disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1601805
DO 10.1183/13993003.01805-2016
VO 50
IS 1
A1 Gerald Burgstaller
A1 Bettina Oehrle
A1 Michael Gerckens
A1 Eric S. White
A1 Herbert B. Schiller
A1 Oliver Eickelberg
YR 2017
UL http://erj.ersjournals.com/content/50/1/1601805.abstract
AB 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.Molecular/biomechanical alterations within ECM in chronic lung diseases direct cellular function/differentiation http://ow.ly/9GrY30c0LJG