ArticleBiomechanics and modeling in mechanobiology2019
Absence of LTBP-3 attenuates the aneurysmal phenotype but not spinal effects on the aorta in Marfan syndrome.
Article in Biomechanics and modeling in mechanobiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 27 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
- Growth arrest of thoracic aortic aneurysms in aging Marfan mice.American journal of physiology. Heart and circulatory physiology · 2026Article
- Transcriptional regulation of postnatal aortic development.Cells & development · 2024Article
- Mapping the unicellular transcriptome of the ascending thoracic aorta to changes in mechanosensing and mechanoadaptation during aging.Aging cell · 2024Article
- TheFrontiers in cardiovascular medicine · 2024Article
- Marfan syndrome: insights from animal models.Frontiers in genetics · 2024Review
- TGFβ-2 haploinsufficiency causes early death in mice with Marfan syndrome.Matrix biology : journal of the International Society for Matrix Biology · 2023Article
- FN (Fibronectin)-Integrin α5 Signaling Promotes Thoracic Aortic Aneurysm in a Mouse Model of Marfan Syndrome.Arteriosclerosis, thrombosis, and vascular biology · 2023Article
- Immune suppressive signaling regulated by latent transforming growth factor beta binding protein 1 promotes metastasis in cervical cancer.Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologica · 2023Article
- G2Φnet: Relating genotype and biomechanical phenotype of tissues with deep learning.PLoS computational biology · 2022Article
- Clonal hematopoiesis of indeterminate potential, DNA methylation, and risk for coronary artery disease.Nature communications · 2022Article
- Adventitial remodeling protects against aortic rupture following late smooth muscle-specific disruption of TGFβ signaling.Journal of the mechanical behavior of biomedical materials · 2021Article
- The Molecular Genetics of Marfan Syndrome.International journal of medical sciences · 2021Review
- Numerical knockouts-In silico assessment of factors predisposing to thoracic aortic aneurysms.PLoS computational biology · 2020Article
- Paradoxical aortic stiffening and subsequent cardiac dysfunction in Hutchinson-Gilford progeria syndrome.Journal of the Royal Society, Interface · 2020Article
- Targetable cellular signaling events mediate vascular pathology in vascular Ehlers-Danlos syndrome.The Journal of clinical investigation · 2020Article
- Association of thoracic spine deformity and cardiovascular disease in a mouse model for Marfan syndrome.PloS one · 2019Article
- Central artery stiffness and thoracic aortopathy.American journal of physiology. Heart and circulatory physiology · 2019Review
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
Abstract
Fibrillin-1 is an elastin-associated glycoprotein that contributes to the long-term fatigue resistance of elastic fibers as well as to the bioavailability of transforming growth factor-beta (TGFβ) in arteries. Altered TGFβ bioavailability and/or signaling have been implicated in aneurysm development in Marfan syndrome (MFS), a multi-system condition resulting from mutations to the gene that encodes fibrillin-1. We recently showed that the absence of the latent transforming growth factor-beta binding protein-3 (LTBP-3) in fibrillin-1-deficient mice attenuates the fragmentation of elastic fibers and focal dilatations that are characteristic of aortic root aneurysms in MFS mice, at least to 12 weeks of age. Here, we show further that the absence of LTBP-3 in this MFS mouse model improves the circumferential mechanical properties of the thoracic aorta, which appears to be fundamental in preventing or significantly delaying aneurysm development. Yet, a spinal deformity either remains or is exacerbated in the absence of LTBP-3 and seems to adversely affect the axial mechanical properties of the thoracic aorta, thus decreasing overall vascular function despite the absence of aneurysmal dilatation. Importantly, because of the smaller size of mice lacking LTBP-3, allometric scaling facilitates proper interpretation of aortic dimensions and thus the clinical phenotype. While this study demonstrates that LTBP-3/TGFβ directly affects the biomechanical function of the thoracic aorta, it highlights that spinal deformities in MFS might indirectly and adversely affect the overall aortic phenotype. There is a need, therefore, to consider together the vascular and skeletal effects in this syndromic disease.
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Registered trials
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