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FGF10 and Human Lung Disease Across the Life Spectrum

Lung diseases impact patients across the lifespan, from infants in the first minutes of life through the aged population. Congenital abnormalities of lung structure can cause lung disease at birth or make adults more susceptible to chronic disease. Continuous inhalation of atmospheric components als...

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Detalles Bibliográficos
Autor principal: Prince, Lawrence S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6220039/
https://www.ncbi.nlm.nih.gov/pubmed/30429870
http://dx.doi.org/10.3389/fgene.2018.00517
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author Prince, Lawrence S.
author_facet Prince, Lawrence S.
author_sort Prince, Lawrence S.
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description Lung diseases impact patients across the lifespan, from infants in the first minutes of life through the aged population. Congenital abnormalities of lung structure can cause lung disease at birth or make adults more susceptible to chronic disease. Continuous inhalation of atmospheric components also requires the lung to be resilient to cellular injury. Fibroblast growth factor 10 (FGF10) regulates multiple stages of structural lung morphogenesis, cellular differentiation, and the response to injury. As a driver of lung airway branching morphogenesis, FGF10 signaling defects during development lead to neonatal lung disease. Alternatively, congenital airway abnormalities attributed to FGF10 mutations increase the risk of chronic airway disease in adulthood. FGF10 also maintains progenitor cell populations in the airway and promotes alveolar type 2 cell expansion and differentiation following injury. Here we review the cellular and molecular mechanisms linking FGF10 to multiple lung diseases, from bronchopulmonary dysplasia in extremely preterm neonates, cystic fibrosis in children, and chronic adult lung disorders. Understanding the connections between FGF10 and lung diseases may lead to exciting new therapeutic strategies.
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spelling pubmed-62200392018-11-14 FGF10 and Human Lung Disease Across the Life Spectrum Prince, Lawrence S. Front Genet Genetics Lung diseases impact patients across the lifespan, from infants in the first minutes of life through the aged population. Congenital abnormalities of lung structure can cause lung disease at birth or make adults more susceptible to chronic disease. Continuous inhalation of atmospheric components also requires the lung to be resilient to cellular injury. Fibroblast growth factor 10 (FGF10) regulates multiple stages of structural lung morphogenesis, cellular differentiation, and the response to injury. As a driver of lung airway branching morphogenesis, FGF10 signaling defects during development lead to neonatal lung disease. Alternatively, congenital airway abnormalities attributed to FGF10 mutations increase the risk of chronic airway disease in adulthood. FGF10 also maintains progenitor cell populations in the airway and promotes alveolar type 2 cell expansion and differentiation following injury. Here we review the cellular and molecular mechanisms linking FGF10 to multiple lung diseases, from bronchopulmonary dysplasia in extremely preterm neonates, cystic fibrosis in children, and chronic adult lung disorders. Understanding the connections between FGF10 and lung diseases may lead to exciting new therapeutic strategies. Frontiers Media S.A. 2018-10-31 /pmc/articles/PMC6220039/ /pubmed/30429870 http://dx.doi.org/10.3389/fgene.2018.00517 Text en Copyright © 2018 Prince. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Genetics
Prince, Lawrence S.
FGF10 and Human Lung Disease Across the Life Spectrum
title FGF10 and Human Lung Disease Across the Life Spectrum
title_full FGF10 and Human Lung Disease Across the Life Spectrum
title_fullStr FGF10 and Human Lung Disease Across the Life Spectrum
title_full_unstemmed FGF10 and Human Lung Disease Across the Life Spectrum
title_short FGF10 and Human Lung Disease Across the Life Spectrum
title_sort fgf10 and human lung disease across the life spectrum
topic Genetics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6220039/
https://www.ncbi.nlm.nih.gov/pubmed/30429870
http://dx.doi.org/10.3389/fgene.2018.00517
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