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Mucins as a New Frontier in Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pulmonary disease with a median survival of 3–5 years after diagnosis. Recent evidence identifies mucins as key effectors in cell growth and tissue remodeling processes compatible with the processes observed in IPF. Mucin...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780288/ https://www.ncbi.nlm.nih.gov/pubmed/31514468 http://dx.doi.org/10.3390/jcm8091447 |
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author | Ballester, Beatriz Milara, Javier Cortijo, Julio |
author_facet | Ballester, Beatriz Milara, Javier Cortijo, Julio |
author_sort | Ballester, Beatriz |
collection | PubMed |
description | Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pulmonary disease with a median survival of 3–5 years after diagnosis. Recent evidence identifies mucins as key effectors in cell growth and tissue remodeling processes compatible with the processes observed in IPF. Mucins are classified in two groups depending on whether they are secreted (secreted mucins) or tethered to cell membranes (transmembrane mucins). Secreted mucins (MUC2, MUC5AC, MUC5B, MUC6-8 and MUC19) are released to the extracellular medium and recent evidence has shown that a promoter polymorphism in the secreted mucin MUC5B is associated with IPF risk. Otherwise, transmembrane mucins (MUC1, MUC3, MUC4, MUC12-17 and MUC20) have a receptor-like structure, sensing the external environment and activating intracellular signal transduction pathways essential for mucosal maintenance and damage repair. In this context, the extracellular domain can be released to the external environment by metalloproteinase action, increased in IPF, thus activating fibrotic processes. For example, several studies have reported increased serum extracellular secreted KL6/MUC1 during IPF acute exacerbation. Moreover, MUC1 and MUC4 overexpression in the main IPF cells has been observed. In this review we summarize the current knowledge of mucins as promising druggable targets for IPF. |
format | Online Article Text |
id | pubmed-6780288 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-67802882019-10-30 Mucins as a New Frontier in Pulmonary Fibrosis Ballester, Beatriz Milara, Javier Cortijo, Julio J Clin Med Review Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pulmonary disease with a median survival of 3–5 years after diagnosis. Recent evidence identifies mucins as key effectors in cell growth and tissue remodeling processes compatible with the processes observed in IPF. Mucins are classified in two groups depending on whether they are secreted (secreted mucins) or tethered to cell membranes (transmembrane mucins). Secreted mucins (MUC2, MUC5AC, MUC5B, MUC6-8 and MUC19) are released to the extracellular medium and recent evidence has shown that a promoter polymorphism in the secreted mucin MUC5B is associated with IPF risk. Otherwise, transmembrane mucins (MUC1, MUC3, MUC4, MUC12-17 and MUC20) have a receptor-like structure, sensing the external environment and activating intracellular signal transduction pathways essential for mucosal maintenance and damage repair. In this context, the extracellular domain can be released to the external environment by metalloproteinase action, increased in IPF, thus activating fibrotic processes. For example, several studies have reported increased serum extracellular secreted KL6/MUC1 during IPF acute exacerbation. Moreover, MUC1 and MUC4 overexpression in the main IPF cells has been observed. In this review we summarize the current knowledge of mucins as promising druggable targets for IPF. MDPI 2019-09-11 /pmc/articles/PMC6780288/ /pubmed/31514468 http://dx.doi.org/10.3390/jcm8091447 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Ballester, Beatriz Milara, Javier Cortijo, Julio Mucins as a New Frontier in Pulmonary Fibrosis |
title | Mucins as a New Frontier in Pulmonary Fibrosis |
title_full | Mucins as a New Frontier in Pulmonary Fibrosis |
title_fullStr | Mucins as a New Frontier in Pulmonary Fibrosis |
title_full_unstemmed | Mucins as a New Frontier in Pulmonary Fibrosis |
title_short | Mucins as a New Frontier in Pulmonary Fibrosis |
title_sort | mucins as a new frontier in pulmonary fibrosis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780288/ https://www.ncbi.nlm.nih.gov/pubmed/31514468 http://dx.doi.org/10.3390/jcm8091447 |
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