Document Detail

The pathogenesis of idiopathic pulmonary fibrosis.
MedLine Citation:
PMID:  20952439     Owner:  NLM     Status:  In-Process    
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an appalling prognosis. The failure of anti-inflammatory therapies coupled with the observation that deranged epithelium overlies proliferative myofibroblasts to form the fibroblastic focus has lead to the emerging concept that IPF is a disease of deregulated epithelial-mesenchymal crosstalk. IPF is triggered by an as yet unidentified alveolar injury that leads to activation of transforming growth factor-β (TGF-β) and alveolar basement membrane disruption. In the presence of persisting injurious pathways, or disrupted repair pathways, activated TGF-β can lead to enhanced epithelial apoptosis and epithelial-to-mesenchymal transition (EMT) as well as fibroblast, and fibrocyte, transformation into myofibroblasts which are resistant to apoptosis. The resulting deposition of excess disrupted matrix by these myofibroblasts leads to the development of IPF.
William R Coward; Gauri Saini; Gisli Jenkins
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-10-15
Journal Detail:
Title:  Therapeutic advances in respiratory disease     Volume:  4     ISSN:  1753-4666     ISO Abbreviation:  Ther Adv Respir Dis     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-11-08     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101316317     Medline TA:  Ther Adv Respir Dis     Country:  England    
Other Details:
Languages:  eng     Pagination:  367-88     Citation Subset:  IM    
Nottingham Respiratory Biomedical Research Unit, Clinical Sciences Building, Nottingham City Campus, Nottingham, UK.
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Grant Support
//Wellcome Trust

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