{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T04:36:43Z","timestamp":1790743003581,"version":"4.1.0"},"reference-count":68,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T00:00:00Z","timestamp":1667433600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-18-CE18-0001"],"award-info":[{"award-number":["ANR-18-CE18-0001"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Association Autour des Williams (France), Soci\u00e9t\u00e9 Fran\u00e7aise de Cardiologie","award":["ANR-18-CE18-0001"],"award-info":[{"award-number":["ANR-18-CE18-0001"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Elastic fibers, made of elastin (90%) and fibrillin-rich microfibrils (10%), are the key extracellular components, which endow the arteries with elasticity. The alteration of elastic fibers leads to cardiovascular dysfunctions, as observed in elastin haploinsufficiency in mice (Eln+\/-) or humans (supravalvular aortic stenosis or Williams\u2013Beuren syndrome). In Eln+\/+ and Eln+\/- mice, we evaluated (arteriography, histology, qPCR, Western blots and cell cultures) the beneficial impact of treatment with a synthetic elastic protein (SEP), mimicking several domains of tropoelastin, the precursor of elastin, including hydrophobic elasticity-related domains and binding sites for elastin receptors. In the aorta or cultured aortic smooth muscle cells from these animals, SEP treatment induced a synthesis of elastin and fibrillin-1, a thickening of the aortic elastic lamellae, a decrease in wall stiffness and\/or a strong trend toward a reduction in the elastic lamella disruptions in Eln+\/- mice. SEP also modified collagen conformation and transcript expressions, enhanced the aorta constrictive response to phenylephrine in several animal groups, and, in female Eln+\/- mice, it restored the normal vasodilatory response to acetylcholine. SEP should now be considered as a biomimetic molecule with an interesting potential for future treatments of elastin-deficient patients with altered arterial structure\/function.<\/jats:p>","DOI":"10.3390\/ijms232113464","type":"journal-article","created":{"date-parts":[[2022,11,4]],"date-time":"2022-11-04T03:28:10Z","timestamp":1667532490000},"page":"13464","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Physiological Impact of a Synthetic Elastic Protein in Arterial Diseases Related to Alterations of Elastic Fibers: Effect on the Aorta of Elastin-Haploinsufficient Male and Female Mice"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8627-1327","authenticated-orcid":false,"given":"Quentin","family":"Bo\u00ebt\u00e9","sequence":"first","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming","family":"Lo","sequence":"additional","affiliation":[{"name":"Institut de Biologie et Chimie des Prot\u00e9ines UMR5305-LBTI, CNRS, Lyon-7, Passage du Vercors, CEDEX 07, 69367 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiao-Ling","family":"Liu","sequence":"additional","affiliation":[{"name":"Institut de Biologie et Chimie des Prot\u00e9ines UMR5305-LBTI, CNRS, Lyon-7, Passage du Vercors, CEDEX 07, 69367 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7333-8526","authenticated-orcid":false,"given":"Guillaume","family":"Vial","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emeline","family":"Lemari\u00e9","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime","family":"Rougelot","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"Steuckardt","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olfa","family":"Harki","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, 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U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Boutin-Paradis","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, U1300, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naima","family":"El Kholti","sequence":"additional","affiliation":[{"name":"Institut de Biologie et Chimie des Prot\u00e9ines UMR5305-LBTI, CNRS, Lyon-7, Passage du Vercors, CEDEX 07, 69367 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurore","family":"Berthier","sequence":"additional","affiliation":[{"name":"Institut de Biologie et Chimie des Prot\u00e9ines UMR5305-LBTI, CNRS, Lyon-7, Passage du Vercors, CEDEX 07, 69367 Lyon, 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