ArticlePloS one2016
Early Impairment of Lung Mechanics in a Murine Model of Marfan Syndrome.
Article in PloS one, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 27 citations in OpenAlex.
- The aorta in Marfan syndrome: from molecular mechanisms to mechanobiological dysfunction.Cardiovascular research · 2026Review
- Active viscoelastic models for cell and tissue mechanics.Royal Society open science · 2024Article
- Effects of aging on the biomechanical properties of the lung extracellular matrix: dependence on tissular stretch.Frontiers in cell and developmental biology · 2024Article
- Innovative three-dimensional models for understanding mechanisms underlying lung diseases: powerful tools for translational research.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- IL11 (Interleukin-11) Causes Emphysematous Lung Disease in a Mouse Model of Marfan Syndrome.Arteriosclerosis, thrombosis, and vascular biology · 2023Article
- The emerging role of NOTCH3 receptor signalling in human lung diseases.Expert reviews in molecular medicine · 2022Review
- Baseline Stiffness Modulates the Non-Linear Response to Stretch of the Extracellular Matrix in Pulmonary Fibrosis.International journal of molecular sciences · 2021Article
- Causal contributors to tissue stiffness and clinical relevance in urology.Communications biology · 2021Review
- Article
- The correlation between spinal and chest wall deformities and pulmonary function in Marfan syndrome.Journal of children's orthopaedics · 2020Article
- Enhanced Notch3 signaling contributes to pulmonary emphysema in a Murine Model of Marfan syndrome.Scientific reports · 2020Article
- Sildenafil Prevents Marfan-Associated Emphysema and Early Pulmonary Artery Dilation in Mice.The American journal of pathology · 2019Article
- Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery.Lung · 2019Article
- Measured pulmonary arterial tissue stiffness is highly sensitive to AFM indenter dimensions.Journal of the mechanical behavior of biomedical materials · 2017Article
- Losartan Attenuates Degradation of Aorta and Lung Tissue Micromechanics in a Mouse Model of Severe Marfan Syndrome.Annals of biomedical engineering · 2016Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
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 (FBN1C1039G/+). The stiffness of the whole lung and of its ECM were measured 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 compliant 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 emphysematous-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 respiratory monitoring could provide early biomarkers for diagnosis and/or follow-up of patients with the Marfan syndrome.
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