mTORC1 activation in lung mesenchyme drives sex- and age-dependent pulmonary structure and function decline

The cellular origins of lymphangioleiomyomatosis (LAM), a rare fatal lung disease, are poorly understood. Here the authors identify a mesenchymal cell hub coordinating the LAM phenotype and develop a LAM mouse model where they investigate the co-operative dysregulation of mTORC1 and WNT growth pathw...

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Autores principales: Kseniya Obraztsova, Maria C. Basil, Ryan Rue, Aravind Sivakumar, Susan M. Lin, Alexander R. Mukhitov, Andrei I. Gritsiuta, Jilly F. Evans, Meghan Kopp, Jeremy Katzen, Annette Robichaud, Elena N. Atochina-Vasserman, Shanru Li, Justine Carl, Apoorva Babu, Michael P. Morley, Edward Cantu, Michael F. Beers, David B. Frank, Edward E. Morrisey, Vera P. Krymskaya
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2020
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Acceso en línea:https://doaj.org/article/920f2bde4ce048d5a6bf73943fc2fe6e
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Sumario:The cellular origins of lymphangioleiomyomatosis (LAM), a rare fatal lung disease, are poorly understood. Here the authors identify a mesenchymal cell hub coordinating the LAM phenotype and develop a LAM mouse model where they investigate the co-operative dysregulation of mTORC1 and WNT growth pathways in the sex- and age-specific changes leading to structural and functional decline.