Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice
The promoter variant rs35705950 confers a gain of function to the MUC5B gene and is the dominant risk factor for idiopathic pulmonary fibrosis. Here the authors show that mice overexpressing Muc5b in distal airspaces show impaired mucociliary clearance and increased susceptibility to bleomycin-induc...
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2018-12-01
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Online Access: | https://doi.org/10.1038/s41467-018-07768-9 |
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doaj-179e7c61d4b74fa68c0ec80470732ff62021-05-11T10:21:43ZengNature Publishing GroupNature Communications2041-17232018-12-019111010.1038/s41467-018-07768-9Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in miceLaura A. Hancock0Corinne E. Hennessy1George M. Solomon2Evgenia Dobrinskikh3Alani Estrella4Naoko Hara5David B. Hill6William J. Kissner7Matthew R. Markovetz8Diane E. Grove Villalon9Matthew E. Voss10Guillermo J. Tearney11Kate S. Carroll12Yunlong Shi13Marvin I. Schwarz14William R. Thelin15Steven M. Rowe16Ivana V. Yang17Christopher M. Evans18David A. Schwartz19Department of Medicine, University of Colorado Denver, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineDepartment of Medicine, University of Alabama at Birmingham, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineMarsico Lung Institute, University of North CarolinaMarsico Lung Institute, University of North CarolinaMarsico Lung Institute, University of North CarolinaParion Sciences, IncParion Sciences, IncWellman Center for Photomedicine and Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts; Department of Pathology, Harvard Medical School, Massachusetts General Hospital, BostonDepartment of Chemistry, The Scripps Research InstituteDepartment of Chemistry, The Scripps Research InstituteDepartment of Medicine, University of Colorado Denver, School of MedicineParion Sciences, IncDepartment of Medicine, University of Alabama at Birmingham, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineDepartment of Medicine, University of Colorado Denver, School of MedicineThe promoter variant rs35705950 confers a gain of function to the MUC5B gene and is the dominant risk factor for idiopathic pulmonary fibrosis. Here the authors show that mice overexpressing Muc5b in distal airspaces show impaired mucociliary clearance and increased susceptibility to bleomycin-induced lung fibrosis, and that both characteristics are reduced by treatment with a mucolytic agent.https://doi.org/10.1038/s41467-018-07768-9 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Laura A. Hancock Corinne E. Hennessy George M. Solomon Evgenia Dobrinskikh Alani Estrella Naoko Hara David B. Hill William J. Kissner Matthew R. Markovetz Diane E. Grove Villalon Matthew E. Voss Guillermo J. Tearney Kate S. Carroll Yunlong Shi Marvin I. Schwarz William R. Thelin Steven M. Rowe Ivana V. Yang Christopher M. Evans David A. Schwartz |
spellingShingle |
Laura A. Hancock Corinne E. Hennessy George M. Solomon Evgenia Dobrinskikh Alani Estrella Naoko Hara David B. Hill William J. Kissner Matthew R. Markovetz Diane E. Grove Villalon Matthew E. Voss Guillermo J. Tearney Kate S. Carroll Yunlong Shi Marvin I. Schwarz William R. Thelin Steven M. Rowe Ivana V. Yang Christopher M. Evans David A. Schwartz Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice Nature Communications |
author_facet |
Laura A. Hancock Corinne E. Hennessy George M. Solomon Evgenia Dobrinskikh Alani Estrella Naoko Hara David B. Hill William J. Kissner Matthew R. Markovetz Diane E. Grove Villalon Matthew E. Voss Guillermo J. Tearney Kate S. Carroll Yunlong Shi Marvin I. Schwarz William R. Thelin Steven M. Rowe Ivana V. Yang Christopher M. Evans David A. Schwartz |
author_sort |
Laura A. Hancock |
title |
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice |
title_short |
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice |
title_full |
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice |
title_fullStr |
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice |
title_full_unstemmed |
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice |
title_sort |
muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice |
publisher |
Nature Publishing Group |
series |
Nature Communications |
issn |
2041-1723 |
publishDate |
2018-12-01 |
description |
The promoter variant rs35705950 confers a gain of function to the MUC5B gene and is the dominant risk factor for idiopathic pulmonary fibrosis. Here the authors show that mice overexpressing Muc5b in distal airspaces show impaired mucociliary clearance and increased susceptibility to bleomycin-induced lung fibrosis, and that both characteristics are reduced by treatment with a mucolytic agent. |
url |
https://doi.org/10.1038/s41467-018-07768-9 |
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