Epithelial stem cell exhaustion in the pathogenesis of idiopathic pulmonary fibrosis

Marco Chilosi, C. Doglioni, B. Murer, V. Poletti

Research output: Contribution to journalArticle

Abstract

New paradigms have been recently proposed in the pathogenesis of idiopathic pulmonary fibrosis (IPF), evidencing that in IPF the cumulative action of an accelerated parenchymal senescence determined by either telomere dysfunction or genetic defects, together with the concurrent noxious activity of tobacco smoking, are able to severely compromise the regenerative potential of parenchymal epithelial stem cells, triggering a cascade of molecular signals and events (scarring, bronchiolar proliferation, abnormal remodelling) eventually leading to severe and irreversible functional impairment. New pathogenic schemes focus on the complex molecular mechanisms driving in a vicious circle the different signalling pathways (e.g. Wnt/ -catenin, TGF-beta, caveolin-1, etc.) potentially involved in epithelial-mesenchymal transition and irreversible lung remodelling.

Original languageEnglish
Pages (from-to)7-18
Number of pages12
JournalSarcoidosis Vasculitis and Diffuse Lung Diseases
Volume27
Issue number1
Publication statusPublished - Jul 2010

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Keywords

  • Epithelial stem cell
  • IPF
  • Senescence
  • Telomere dysfunction
  • Wnt-signalling-pathway

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Immunology and Allergy
  • Internal Medicine

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