Role of the WNT/beta-catenin signal pathway in idiopathic and experimental pulmonary fibrosis
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Abstract
Human idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease of unknown origin, which is refractory to any currently available therapy. It is characterised by initial alveolar epithelial cell injury and hyperplasia, enhanced fibroblast/myofibroblast proliferation and activation and increased deposition of extracellular matrix (ECM) in the lung interstitium. These key features, ultimately lead to architectural distortion of the normal lung parenchyma and due to severe loss of function, to respiratory failure. However, the molecular mechanisms underlying alveolar epithelial type II (ATII) cell dysfunction are still poorly understood.This study is based on the hypothesis that the WNT/beta-catenin signalling pathway, which is essential for organ development, is aberrantly activated in ATII cells in idiopathic and experimental pulmonary fibrosis. The role of canonical WNT signalling was elucidated by determining the expression, function and activity of the pathway.In summary, this study shows that WNT signalling is expressed and localised in idiopathic and experimental pulmonary fibrosis, in particular in alveolar ATII cells. Reversal and/or inhibition predominantly of the WNT/beta-catenin signalling pathway, but equally the use of neutralising antibodies or inhibitors of IL1beta/IL6 may represent a valid therapeutic option in human lung fibrosis.Link to publications or other datasets
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Giessen : VVB Laufersweiler