Cargando…
What have we learned from basic science studies on idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis is a fatal age-related lung disease characterised by progressive and irreversible scarring of the lung. Although the details are not fully understood, there has been tremendous progress in understanding the pathogenesis of idiopathic pulmonary fibrosis, which has led to...
Autores principales: | , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
European Respiratory Society
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488501/ https://www.ncbi.nlm.nih.gov/pubmed/31511255 http://dx.doi.org/10.1183/16000617.0029-2019 |
_version_ | 1784792672301481984 |
---|---|
author | Yanagihara, Toyoshi Sato, Seidai Upagupta, Chandak Kolb, Martin |
author_facet | Yanagihara, Toyoshi Sato, Seidai Upagupta, Chandak Kolb, Martin |
author_sort | Yanagihara, Toyoshi |
collection | PubMed |
description | Idiopathic pulmonary fibrosis is a fatal age-related lung disease characterised by progressive and irreversible scarring of the lung. Although the details are not fully understood, there has been tremendous progress in understanding the pathogenesis of idiopathic pulmonary fibrosis, which has led to the identification of many new potential therapeutic targets. In this review we discuss several of these advances with a focus on genetic susceptibility and cellular senescence primarily affecting epithelial cells, activation of profibrotic pathways, disease-enhancing fibrogenic cell types and the role of the remodelled extracellular matrix. |
format | Online Article Text |
id | pubmed-9488501 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | European Respiratory Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-94885012022-11-14 What have we learned from basic science studies on idiopathic pulmonary fibrosis? Yanagihara, Toyoshi Sato, Seidai Upagupta, Chandak Kolb, Martin Eur Respir Rev Review Idiopathic pulmonary fibrosis is a fatal age-related lung disease characterised by progressive and irreversible scarring of the lung. Although the details are not fully understood, there has been tremendous progress in understanding the pathogenesis of idiopathic pulmonary fibrosis, which has led to the identification of many new potential therapeutic targets. In this review we discuss several of these advances with a focus on genetic susceptibility and cellular senescence primarily affecting epithelial cells, activation of profibrotic pathways, disease-enhancing fibrogenic cell types and the role of the remodelled extracellular matrix. European Respiratory Society 2019-09-11 /pmc/articles/PMC9488501/ /pubmed/31511255 http://dx.doi.org/10.1183/16000617.0029-2019 Text en Copyright ©ERS 2019. https://creativecommons.org/licenses/by-nc/4.0/This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. |
spellingShingle | Review Yanagihara, Toyoshi Sato, Seidai Upagupta, Chandak Kolb, Martin What have we learned from basic science studies on idiopathic pulmonary fibrosis? |
title | What have we learned from basic science studies on idiopathic pulmonary fibrosis? |
title_full | What have we learned from basic science studies on idiopathic pulmonary fibrosis? |
title_fullStr | What have we learned from basic science studies on idiopathic pulmonary fibrosis? |
title_full_unstemmed | What have we learned from basic science studies on idiopathic pulmonary fibrosis? |
title_short | What have we learned from basic science studies on idiopathic pulmonary fibrosis? |
title_sort | what have we learned from basic science studies on idiopathic pulmonary fibrosis? |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488501/ https://www.ncbi.nlm.nih.gov/pubmed/31511255 http://dx.doi.org/10.1183/16000617.0029-2019 |
work_keys_str_mv | AT yanagiharatoyoshi whathavewelearnedfrombasicsciencestudiesonidiopathicpulmonaryfibrosis AT satoseidai whathavewelearnedfrombasicsciencestudiesonidiopathicpulmonaryfibrosis AT upaguptachandak whathavewelearnedfrombasicsciencestudiesonidiopathicpulmonaryfibrosis AT kolbmartin whathavewelearnedfrombasicsciencestudiesonidiopathicpulmonaryfibrosis |