ArticleProceedings of the National Academy of Sciences of the United States of America2010
Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 81 papers.
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81 citing papers in PubMed, 187 citations in OpenAlex.
- The aorta in Marfan syndrome: from molecular mechanisms to mechanobiological dysfunction.Cardiovascular research · 2026Review
- Hypertension drives thoracic aortic aneurysm and dissection in male, but not female, Marfan mice.Journal of the mechanical behavior of biomedical materials · 2026Article
- Fibronectin-induced overactivation of αNature communications · 2026Article
- Association between ocular manifestations and incident cardiovascular comorbidities in Ehlers Danlos Syndrome.Eye (London, England) · 2026Article
- Mechanotransduction in Marfan Syndrome and Related Aortic Disorders: Insights from Transcriptomic Analyses.Genes · 2026Review
- Valve-Dependent Regional Heterogeneity of Wall Mechanics and Collagen Remodeling in Ascending Thoracic Aortic Aneurysms.International journal of molecular sciences · 2026Article
- Nano-mechanical mapping of human and porcine abdominal aortic aneurysm.Journal of the mechanical behavior of biomedical materials · 2026Article
- Dissection Propagation via Avalanches in Human Descending Thoracic Aorta: Effect of Aging.Acta biomaterialia · 2025Article
- Right Ventricular Stiffening and Anisotropy Alterations in Pulmonary Hypertension: Mechanisms and Relations to Right Heart Failure.Journal of the American Heart Association · 2025Article
- Mitochondrial NADNature cardiovascular research · 2025Article
- Sensitivity to Aortic Rupture in Hereditary Aortic Diseases.Thrombosis and haemostasis · 2025Article
- Sacrificial Templating for Accelerating Clinical Translation of Engineered Organs.ACS biomaterials science & engineering · 2025Review
- Distribution and Maturity of Medial Collagen Fibers in Thoracoabdominal Post-Dissection Aortic Aneurysms: A Comparative Study of Marfan and Non-Marfan Patients.International journal of molecular sciences · 2024Article
- In Vivo Quantification of Ascending Thoracic Aortic Aneurysm Wall Stretch Using MRI: Relationship to Repair Threshold Diameter and Ex Vivo Wall Failure Behavior.Journal of biomechanical engineering · 2024Article
- Age- and sex-specific biomechanics and extracellular matrix remodeling of the ascending aorta in a mouse model of severe Marfan syndrome.American journal of physiology. Heart and circulatory physiology · 2024Article
- Investigating the influence of collagen cross-linking on mechanical properties of thoracic aortic tissue.Frontiers in bioengineering and biotechnology · 2024Article
- Evaluation of aortic wall strength in human immunodeficiency virus associated thoracic ascending aortic aneurysm: a pilot study.Cardiovascular diagnosis and therapy · 2023Article
- Effect of aging on the biaxial mechanical behavior of human descending thoracic aorta: Experiments and constitutive modeling considering collagen crosslinking.Journal of the mechanical behavior of biomedical materials · 2023Article
- Impact of adipose-derived stem cells on aortic tensile strength in a model of abdominal aortic aneurysm.American heart journal plus : cardiology research and practice · 2023Article
- Different hemodynamic factors cause the occurrence of superior mesenteric atherosclerotic stenosis and superior mesenteric artery dissection.Frontiers in cardiovascular medicine · 2023Article
21 more citing papers are in PubMed but not listed here.
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
An aneurysm of the aorta is a common pathology characterized by segmental weakening of the artery. Although it is generally accepted that the vessel-wall weakening is caused by an impaired collagen metabolism, a clear association has been demonstrated only for rare syndromes such as the vascular type Ehlers-Danlos syndrome. Here we show that vessel-wall failure in growing aneurysms of patients who have aortic abdominal aneurysm (AAA) or Marfan syndrome is not related to a collagen defect at the molecular level. On the contrary our findings indicate similar (Marfan) or even higher collagen concentrations (AAA) and increased collagen cross-linking in the aneurysms. Using 3D confocal imaging we show that the two conditions are associated with profound defects in collagen microarchitecture. Reconstructions of normal vessel wall show that adventitial collagen fibers are organized in a loose braiding of collagen ribbons. These ribbons encage the vessel, allowing the vessel to dilate easily but preventing overstretching. AAA and aneurysms in Marfan syndrome show dramatically altered collagen architectures with loss of the collagen knitting. Evaluations of the functional characteristics by atomic force microscopy showed that the wall has lost its ability to stretch easily and revealed a second defect: although vascular collagen in normal aortic wall behaves as a coherent network, in AAA and Marfan tissues it does not. As result, mechanical forces loaded on individual fibers are not distributed over the tissue. These studies demonstrate that the mechanical properties of tissue are strongly influenced by collagen microarchitecture and that perturbations in the collagen networks may lead to mechanical failure.
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