Article
Matrix abnormalities in pulmonary fibrosis.
European respiratory review : an official journal of the European Respiratory Society - 30 Jun 2018
Upagupta Chandak, Shimbori Chiko, Alsilmi Rahmah, Kolb Martin
Abstract excerpt
Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive disease, marked by excessive scarring, which leads to increased tissue stiffness, loss in lung function and ultimately death. IPF is characterised by progressive fibroblast and myofibroblast proliferation, and extensive deposition of extracellular matrix (ECM). Myofibroblasts play a key role in ECM deposition. Transforming growth factor (TGF)-β1 is...
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