{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T08:30:46Z","timestamp":1776933046210,"version":"3.51.2"},"reference-count":55,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2020,9,24]],"date-time":"2020-09-24T00:00:00Z","timestamp":1600905600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100007229","name":"Bijzonder Onderzoeksfonds UGent","doi-asserted-by":"publisher","award":["01M01108"],"award-info":[{"award-number":["01M01108"]}],"id":[{"id":"10.13039\/501100007229","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fonds voor hartchirurgie","award":["489644"],"award-info":[{"award-number":["489644"]}]},{"DOI":"10.13039\/501100003130","name":"Fonds Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","award":["Postdoctoral scholarship Marjolijn Renard"],"award-info":[{"award-number":["Postdoctoral scholarship Marjolijn Renard"]}],"id":[{"id":"10.13039\/501100003130","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003130","name":"Fonds Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","award":["Senior Clinical Investigator scholarship Julie De Backer"],"award-info":[{"award-number":["Senior Clinical Investigator scholarship Julie De Backer"]}],"id":[{"id":"10.13039\/501100003130","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010665","name":"H2020 Marie Sk\u0142odowska-Curie Actions","doi-asserted-by":"publisher","award":["794365"],"award-info":[{"award-number":["794365"]}],"id":[{"id":"10.13039\/100010665","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Patients with Marfan syndrome (MFS), a connective tissue disorder caused by pathogenic variants in the gene encoding the extracellular matrix protein fibrillin-1, have an increased prevalence of primary cardiomyopathy, arrhythmias, and sudden cardiac death. We have performed an in-depth in vivo and ex vivo study of the cardiac phenotype of Fbn1mgR\/mgR mice, an established mouse model of MFS with a severely reduced expression of fibrillin-1. Using ultrasound measurements, we confirmed the presence of aortic dilatation and observed cardiac diastolic dysfunction in male Fbn1mgR\/mgR mice. Upon post-mortem examination, we discovered that the mutant mice consistently presented myocardial lesions at the level of the right ventricular free wall, which we characterized as spontaneous pseudoaneurysms. Histological investigation demonstrated a decrease in myocardial compaction in the MFS mouse model. Furthermore, continuous 24 h electrocardiographic analysis showed a decreased heart rate variability and an increased prevalence of extrasystolic arrhythmic events in Fbn1mgR\/mgR mice compared to wild-type littermates. Taken together, in this paper we document a previously unreported cardiac phenotype in the Fbn1mgR\/mgR MFS mouse model and provide a detailed characterization of the cardiac dysfunction and rhythm disorders which are caused by fibrillin-1 deficiency. These findings highlight the wide spectrum of cardiac manifestations of MFS, which might have implications for patient care.<\/jats:p>","DOI":"10.3390\/ijms21197024","type":"journal-article","created":{"date-parts":[[2020,9,24]],"date-time":"2020-09-24T08:33:03Z","timestamp":1600936383000},"page":"7024","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Spontaneous Right Ventricular Pseudoaneurysms and Increased Arrhythmogenicity in a Mouse Model of Marfan Syndrome"],"prefix":"10.3390","volume":"21","author":[{"given":"Felke","family":"Steijns","sequence":"first","affiliation":[{"name":"Center for Medical Genetics, Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marjolijn","family":"Renard","sequence":"additional","affiliation":[{"name":"Center for Medical Genetics, Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marine","family":"Vanhomwegen","sequence":"additional","affiliation":[{"name":"Center for Medical Genetics, Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Vermassen","sequence":"additional","affiliation":[{"name":"Center for Medical Genetics, Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4261-3983","authenticated-orcid":false,"given":"Jana","family":"Desloovere","sequence":"additional","affiliation":[{"name":"4BRAIN, Department of Head and Skin, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8939-0169","authenticated-orcid":false,"given":"Robrecht","family":"Raedt","sequence":"additional","affiliation":[{"name":"4BRAIN, Department of Head and Skin, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lars E.","family":"Larsen","sequence":"additional","affiliation":[{"name":"4BRAIN, Department of Head and Skin, Ghent University, 9000 Ghent, Belgium"},{"name":"Institute Biomedical Technology, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00e1t\u00e9 I.","family":"T\u00f3th","sequence":"additional","affiliation":[{"name":"Institute Biomedical Technology, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8878-1507","authenticated-orcid":false,"given":"Julie","family":"De Backer","sequence":"additional","affiliation":[{"name":"Center for Medical Genetics, Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium"},{"name":"Department of Cardiology, Ghent University Hospital, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9241-5980","authenticated-orcid":false,"given":"Patrick","family":"Sips","sequence":"additional","affiliation":[{"name":"Center for Medical Genetics, Department of Biomolecular Medicine, Ghent University, 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,9,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1161\/CIRCRESAHA.114.302533","article-title":"Myocardial Extracellular Matrix an Ever-Changing and Diverse Entity","volume":"114","author":"Rienks","year":"2014","journal-title":"Circ. 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