Early Impairment of Lung Mechanics in a Murine Model of Marfan Syndrome.

Early morbidity and mortality in patients with Marfan syndrome (MFS) -a connective tissue disease caused by mutations in fibrillin-1 gene- are mainly caused by aorta aneurysm and rupture. However, the increase in the life expectancy of MFS patients recently achieved by reparatory surgery promotes cl...

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Bibliographic Details
Main Authors: Juan J Uriarte, Thayna Meirelles, Darya Gorbenko Del Blanco, Paula N Nonaka, Noelia Campillo, Elisabet Sarri, Daniel Navajas, Gustavo Egea, Ramon Farré
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2016-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0152124