ArticleCardiovascular research2011
Haploinsufficiency of the murine Col3a1 locus causes aortic dissection: a novel model of the vascular type of Ehlers-Danlos syndrome.
Article in Cardiovascular research, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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37 citing papers in PubMed, 81 citations in OpenAlex.
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- Cardiac health, type I collagen, and aging in theAmerican journal of physiology. Heart and circulatory physiology · 2025Article
- Map2k6 is a potent genetic modifier of arterial rupture in vascular Ehlers-Danlos syndrome mice.JCI insight · 2025Article
- Modeling Thoracic Aortic Dissection Using Patient-Specific iPSCs Reveals VSMC Dysfunction and Extracellular Matrix Dysregulation.Stem cells international · 2025Article
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- Vascular Ehlers-Danlos Syndrome: A Comprehensive Natural History Study in a Dutch National Cohort of 142 Patients.Circulation. Genomic and precision medicine · 2024Article
- Comparison and Evaluation of Two Combination Modes of Angiotensin for Establishing Murine Aortic Dissection Models.Journal of cardiovascular translational research · 2023Article
- Molecular and Cellular Mechanisms Involved in Aortic Wall Aneurysm Development.Diagnostics (Basel, Switzerland) · 2023Review
- Novel variants identified in a three‑generation family with concomitant exotropia.Experimental and therapeutic medicine · 2022Article
- Fibrillar Collagen Variants in Spontaneous Coronary Artery Dissection.JAMA cardiology · 2022Article
- Comparative therapeutic strategies for preventing aortic rupture in a mouse model of vascular Ehlers-Danlos syndrome.PLoS genetics · 2022Article
- Four decades in the making: Collagen III and mechanisms of vascular Ehlers Danlos Syndrome.Matrix biology plus · 2021Review
- Animal Models of Ehlers-Danlos Syndromes: Phenotype, Pathogenesis, and Translational Potential.Frontiers in genetics · 2021Review
- Targetable cellular signaling events mediate vascular pathology in vascular Ehlers-Danlos syndrome.The Journal of clinical investigation · 2020Article
- Genetic Deletion of Socs3 in Smooth Muscle Cells Ameliorates Aortic Dissection in Mice.JACC. Basic to translational science · 2020Article
- Recent Advances in Biomechanical Characterization of Thoracic Aortic Aneurysms.Frontiers in cardiovascular medicine · 2020Review
- Extracellular matrix, regional heterogeneity of the aorta, and aortic aneurysm.Experimental & molecular medicine · 2019Review
- Review
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10 authors at 4 institutions in 1 country.
Funding
Abstract
aimsThe vascular type of Ehlers-Danlos syndrome (EDS IV) is an autosomal-dominant disorder characterized by thin translucent skin and extensive bruising. Patients with EDS IV have reduced life expectancy (median 45-50 years) due to spontaneous rupture of arteries (particularly large arteries) or bowel. EDS IV results from mutation of the COL3A1 gene, which encodes the pro-α(1) chains of type III collagen that is secreted into the extracellular matrix, e.g. by smooth muscle cells. A mouse model of EDS IV produced by targeted ablation of Col3a1 has been of limited use as only 10% of homozygous animals survive to adulthood, whereas heterozygous animals do not die from arterial rupture. We report a novel, exploitable model of EDS IV in a spontaneously generated mouse line. METHODS AND
resultsMice were identified by predisposition to sudden, unexpected death from dissection of the thoracic aorta. Aortic dissection inheritance was autosomal-dominant, presented at an early age (median, 6 weeks) with incomplete penetrance, and had a similar sex ratio bias as EDS IV (2:1, male:female). Molecular genetic analysis demonstrated that the causal mutation is a spontaneous 185 kb deletion, including the promoter region and exons 1-39, of the Col3a1 gene. As in EDS IV, aortic dissection was not associated with elevated blood pressure, aneurysm formation, or infection, but may result from aberrant collagen fibrillogenesis within the aortic wall.
conclusionThis novel, exploitable mouse line that faithfully models the vascular aspects of human EDS IV provides an important new tool for advancing understanding of EDS IV and of aortic dissection in general.
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