Nature Communications (Nov 2020)
mTORC1 activation in lung mesenchyme drives sex- and age-dependent pulmonary structure and function decline
- 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
Affiliations
- Kseniya Obraztsova
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Maria C. Basil
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Ryan Rue
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Aravind Sivakumar
- Children Hospital of Philadelphia
- Susan M. Lin
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Alexander R. Mukhitov
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Andrei I. Gritsiuta
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Jilly F. Evans
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Meghan Kopp
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Jeremy Katzen
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Annette Robichaud
- SCIREQ Inc.
- Elena N. Atochina-Vasserman
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- Shanru Li
- Lung Biology Institute, University of Pennsylvania
- Justine Carl
- Lung Biology Institute, University of Pennsylvania
- Apoorva Babu
- Lung Biology Institute, University of Pennsylvania
- Michael P. Morley
- Lung Biology Institute, University of Pennsylvania
- Edward Cantu
- Lung Biology Institute, University of Pennsylvania
- Michael F. Beers
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- David B. Frank
- Lung Biology Institute, University of Pennsylvania
- Edward E. Morrisey
- Lung Biology Institute, University of Pennsylvania
- Vera P. Krymskaya
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania
- DOI
- https://doi.org/10.1038/s41467-020-18979-4
- Journal volume & issue
-
Vol. 11,
no. 1
pp. 1 – 15
Abstract
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.
