Extracellular matrix in lung development, homeostasis and disease

The lung's unique extracellular matrix (ECM), while providing structural support for cells, is critical in the regulation of developmental organogenesis, homeostasis and injury-repair responses. The ECM, via biochemical or biomechanical cues, regulates diverse cell functions, fate and phenotype. The composition and function of lung ECM become markedly deranged in pathological tissue remodeling. ECM-based therapeutics and bioengineering approaches represent promising novel strategies for regeneration/repair of the lung and treatment of chronic lung diseases. In this review, we assess the current state of lung ECM biology, including fundamental advances in ECM composition, dynamics, topography, and biomechanics; the role of the ECM in normal and aberrant lung development, adult lung diseases and autoimmunity; and ECM in the regulation of the stem cell niche. We identify opportunities to advance the field of lung ECM biology and provide a set recommendations for research priorities to advance knowledge that would inform novel approaches to the pathogenesis, diagnosis, and treatment of chronic lung diseases. •Recent advances in ECM composition, dynamics, topography, and biomechanics•ECM in normal lung development and aberrant development (BPD)•ECM dynamics and altered deposition in adult lung diseases, namely COPD and IPF•ECM in inflammation/autoimmunity; and maintenance of the stem cell niche•ECM-based therapeutics for chronic lung diseases•Critical questions and emerging opportunities in lung ECM biology research