{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T19:48:07Z","timestamp":1790192887141,"version":"4.1.0"},"reference-count":107,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,5,11]],"date-time":"2021-05-11T00:00:00Z","timestamp":1620691200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Ministerio de Ciencia e Innovaci\u00f3n (MCIN), Gobierno de Espa\u00f1a","award":["PID2019-108489RB-I00"],"award-info":[{"award-number":["PID2019-108489RB-I00"]}]},{"name":"Instituto de Salud Carlos III (ISCIII)","award":["AC17\/00067"],"award-info":[{"award-number":["AC17\/00067"]}]},{"DOI":"10.13039\/100002287","name":"Progeria Research Foundation","doi-asserted-by":"publisher","award":["PRF 2019\u201377"],"award-info":[{"award-number":["PRF 2019\u201377"]}],"id":[{"id":"10.13039\/100002287","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fundaci\u00f3n Marat\u00f3 TV3","award":["38\/C\/2020"],"award-info":[{"award-number":["38\/C\/2020"]}]},{"name":"Comunidad Aut\u00f3noma de Madrid","award":["2017-T1\/BMD-5247"],"award-info":[{"award-number":["2017-T1\/BMD-5247"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease that recapitulates many symptoms of physiological aging and precipitates death. Patients develop severe vascular alterations, mainly massive vascular smooth muscle cell loss, vessel stiffening, calcification, fibrosis, and generalized atherosclerosis, as well as electrical, structural, and functional anomalies in the heart. As a result, most HGPS patients die of myocardial infarction, heart failure, or stroke typically during the first or second decade of life. No cure exists for HGPS, and therefore it is of the utmost importance to define the mechanisms that control disease progression in order to develop new treatments to improve the life quality of patients and extend their lifespan. Since the discovery of the HGPS-causing mutation, several animal models have been generated to study multiple aspects of the syndrome and to analyze the contribution of different cell types to the acquisition of the HGPS-associated cardiovascular phenotype. This review discusses current knowledge about cardiovascular features in HGPS patients and animal models and the molecular and cellular mechanisms through which progerin causes cardiovascular disease.<\/jats:p>","DOI":"10.3390\/cells10051157","type":"journal-article","created":{"date-parts":[[2021,5,11]],"date-time":"2021-05-11T04:05:32Z","timestamp":1620705932000},"page":"1157","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":49,"title":["Molecular and Cellular Mechanisms Driving Cardiovascular Disease in Hutchinson-Gilford Progeria Syndrome: Lessons Learned from Animal Models"],"prefix":"10.3390","volume":"10","author":[{"given":"Ignacio","family":"Benedicto","sequence":"first","affiliation":[{"name":"Centro Nacional de Investigaciones Cardiovasculares (CNIC), 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatriz","family":"Dorado","sequence":"additional","affiliation":[{"name":"Centro Nacional de Investigaciones Cardiovasculares (CNIC), 28029 Madrid, Spain"},{"name":"CIBER en Enfermedades Cardiovasculares (CIBER-CV), Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0125-7209","authenticated-orcid":false,"given":"Vicente","family":"Andr\u00e9s","sequence":"additional","affiliation":[{"name":"Centro Nacional de Investigaciones Cardiovasculares (CNIC), 28029 Madrid, Spain"},{"name":"CIBER en Enfermedades Cardiovasculares (CIBER-CV), Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.ceb.2016.12.005","article-title":"A-type lamins and cardiovascular disease in premature aging syndromes","volume":"46","author":"Dorado","year":"2017","journal-title":"Curr. 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