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...

Full description

Bibliographic Details
Main Authors:	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
Format:	Article
Language:	English
Published:	Nature Publishing Group 2018-12-01
Series:	Nature Communications
Online Access:	https://doi.org/10.1038/s41467-018-07768-9

id	doaj-179e7c61d4b74fa68c0ec80470732ff6
record_format	Article
spelling	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
work_keys_str_mv	AT lauraahancock muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT corinneehennessy muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT georgemsolomon muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT evgeniadobrinskikh muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT alaniestrella muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT naokohara muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT davidbhill muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT williamjkissner muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT matthewrmarkovetz muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT dianeegrovevillalon muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT matthewevoss muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT guillermojtearney muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT katescarroll muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT yunlongshi muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT marvinischwarz muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT williamrthelin muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT stevenmrowe muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT ivanavyang muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT christophermevans muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice AT davidaschwartz muc5boverexpressioncausesmucociliarydysfunctionandenhanceslungfibrosisinmice
_version_	1721448247092838400

Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice

Similar Items