{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T13:56:08Z","timestamp":1783605368386,"version":"3.55.0"},"reference-count":60,"publisher":"Georg Thieme Verlag KG","issue":"02","license":[{"start":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T00:00:00Z","timestamp":1719792000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Isaac Dreyfus-Bernheim Stiftung and the Fondation pour la Recherche et le Traitement M\u00e9dical"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health NHLBI","doi-asserted-by":"crossref","award":["K01 HL149984"],"award-info":[{"award-number":["K01 HL149984"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"name":"JSPS KAKENHI","award":["JP20H03762"],"award-info":[{"award-number":["JP20H03762"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:p>Objective\u2003Hereditary aortic diseases (hADs) increase the risk of aortic dissections and ruptures. Recently, we have established an objective approach to measure the rupture force of the murine aorta, thereby explaining the outcomes of clinical studies and assessing the added value of approved drugs in vascular Ehlers\u2013Danlos syndrome (vEDS). Here, we applied our approach to six additional mouse hAD models.<\/jats:p>\n                  <jats:p>Material and Methods\u2003We used two mouse models (Fbn1C1041G\n             and Fbn1mgR\n            ) of Marfan syndrome (MFS) as well as one smooth-muscle-cell-specific knockout (SMKO) of Efemp2 and three CRISPR\/Cas9-engineered knock-in models (Ltbp1, Mfap4, and Timp1). One of the two MFS models was subjected to 4-week-long losartan treatment. Per mouse, three rings of the thoracic aorta were prepared, mounted on a tissue puller, and uniaxially stretched until rupture.<\/jats:p>\n                  <jats:p>Results\u2003The aortic rupture force of the SMKO and both MFS models was significantly lower compared with wild-type mice but in both MFS models higher than in mice modeling vEDS. In contrast, the Ltbp1, Mfap4, and Timp1 knock-in models presented no impaired aortic integrity. As expected, losartan treatment reduced aneurysm formation but surprisingly had no impact on the aortic rupture force of our MFS mice.<\/jats:p>\n                  <jats:p>Conclusion\u2003Our read-out system can characterize the aortic biomechanical integrity of mice modeling not only vEDS but also related hADs, allowing the aortic-rupture-force-focused comparison of mouse models. Furthermore, aneurysm progression alone may not be a sufficient read-out for aortic rupture, as antihypertensive drugs reducing aortic dilatation might not strengthen the weakened aortic wall. Our results may enable identification of improved medical therapies of hADs.<\/jats:p>","DOI":"10.1055\/s-0044-1787957","type":"journal-article","created":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T19:18:36Z","timestamp":1719861516000},"page":"142-152","source":"Crossref","is-referenced-by-count":5,"title":["Novel Insights into the Aortic Mechanical Properties of Mice Modeling Hereditary Aortic Diseases"],"prefix":"10.1055","volume":"125","author":[{"given":"Nicolo","family":"Dubacher","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Genetics and Gene Diagnostics, Swiss Foundation for People with Rare Diseases, Schlieren-Zurich, Switzerland"},{"name":"Translational Cardiovascular Technologies, Department of Health Sciences, ETH Zurich, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaori","family":"Sugiyama","sequence":"additional","affiliation":[{"name":"Institute for Advanced Research of Biosystem Dynamics, Research Institute for Science and Engineering, Waseda University, Tokyo, Japan"},{"name":"Life Science Center for Survival Dynamics, Tsukuba Advanced Research Alliance, University of Tsukuba, Tsukuba, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey D.","family":"Smith","sequence":"additional","affiliation":[{"name":"Saha Cardiovascular Research Center, University of Kentucky, Lexington, Kentucky, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Nussbaumer","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Genetics and Gene Diagnostics, Swiss Foundation for People with Rare Diseases, Schlieren-Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1t\u00e9","family":"Csonka","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Genetics and Gene Diagnostics, Swiss Foundation for People with Rare Diseases, Schlieren-Zurich, 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Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Lamberti","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Genetics and Gene Diagnostics, Swiss Foundation for People with Rare Diseases, Schlieren-Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janine","family":"Meienberg","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Genetics and Gene Diagnostics, Swiss Foundation for People with Rare Diseases, Schlieren-Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiromi","family":"Yanagisawa","sequence":"additional","affiliation":[{"name":"Life Science Center for Survival Dynamics, Tsukuba Advanced Research Alliance, University of Tsukuba, Tsukuba, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary B.","family":"Sheppard","sequence":"additional","affiliation":[{"name":"Department of Family and Community 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