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Title: Early impairment of lung mechanics in a murine model of Marfan syndrome
Author: Uriarte, Juan José
Meirelles, Thayna
Gorbenko del Blanco, Darya
Nonaka, Paula Naomi
Campillo, Noelia
Sarri Plans, Elisabet
Navajas Navarro, Daniel
Egea Guri, Gustavo
Farré Ventura, Ramon
Keywords: Malalties hereditàries
Aparell respiratori
Genetic diseases
Respiratory organs
Issue Date: 22-Mar-2016
Publisher: Public Library of Science (PLoS)
Abstract: 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 knowledge of how this connective tissue disease modifies lung mechanics is scarce. Hence, we assessed whether the stiffness of the whole lung and of its extracellular matrix (ECM) is affected in a well-characterized MFS mouse model (FBN1 C1039G/+ ). The stiffness of the whole lung and of its ECM were mea- sured by conventional mechanical ventilation and atomic force microscopy, respectively. We studied 5-week and 9-month old mice, whose ages are representative of early and late stages of the disease. At both ages, the lungs of MFS mice were significantly more compli- ant than in wild type (WT) mice. By contrast, no significant differences were found in local lung ECM stiffness. Moreover, histopathological lung evaluation showed a clear emphyse- matous-like pattern in MFS mice since alveolar space enlargement was significantly increased compared with WT mice. These data suggest that the mechanism explaining the increased lung compliance in MFS is not a direct consequence of reduced ECM stiffness, but an emphysema-like alteration in the 3D structural organization of the lung. Since lung alterations in MFS are almost fully manifested at an early age, it is suggested that respira- tory monitoring could provide early biomarkers for diagnosis and/or follow-up of patients with the Marfan syndrome
Note: Reproducció del document publicat a:
It is part of: PLoS One, 2016, vol. 11, num. 3, p. e0152124
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ISSN: 1932-6203
Appears in Collections:Articles publicats en revistes (Biomedicina)

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