Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice

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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Autores principales: 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
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2018
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Science
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Acceso en línea:https://doaj.org/article/179e7c61d4b74fa68c0ec80470732ff6
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Sumario: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.

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