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Article

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

PloS one - 1 Jan 2016

Uriarte Juan J, Meirelles Thayna, Gorbenko Del Blanco Darya, Nonaka Paula N, Campillo Noelia, Sarri Elisabet, Navajas Daniel, Egea Gustavo, Farré Ramon

Abstract excerpt

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 clinical manifestations in other organs. Although some studies have reported respiratory alterations in MFS, our...

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Identifiers

PMID: 27003297
DOI: 10.1371/journal.pone.0152124
PMCID: PMC4803219

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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