41 The multi-faceted extracellular matrix: unlocking its secrets for understanding the perpetuation of lung fibrosis CONCLUSION There is an urgent need for establishing effective processes for diagnosing patients with fibrotic lung diseases, and an even greater need for being able to accurately identify the underlying pathological cause to then be able to effectively manage these patients, without causing further harm. The ultimate goal is to enable the development of therapeutic approaches that are able to reverse the destructive changes in the lung tissue to regenerate effective gas exchange units and to return the longevity and quality of life for these patients. Thinking about the ECM as an active contributory element within the disease process has the potential to provide far reaching opportunities for novel advances in identifying disease-modifying mechanisms for pulmonary fibrosis. A consideration of the diversity of “hidden” changes within the ECM milieu that go far beyond the well-recognized changes in the composition and amount of ECM in a fibrotic lesion within lung tissues augments the novel directions that can be pursued when searching for future therapeutic targets (Figure 3). Future studies using emerging novel in vitro models that incorporate dimensionality and mechanical elements that exist in the lung, coupled with stateof-the-art transcriptomic and spatial proteomic profiling of fibrotic lung tissues, have the potential to ensure exciting developments in our understanding and management of pulmonary fibrosis in the near future. Figure 3: ECM changes and potential therapeutic targeting sites/points. ECM: Extracellular matrix; LOX: Lysyl oxidase; LOXL: Lysyl oxidase-like; TG: Transglutaminase. 2
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