{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T01:03:26Z","timestamp":1792717406180,"version":"4.5.0"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"12","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["ATVB"],"published-print":{"date-parts":[[2009,12]]},"abstract":"<jats:p>\n            <jats:bold>\n              <jats:italic>Objective\u2014<\/jats:italic>\n            <\/jats:bold>\n            Even though elastin and fibrillin-1 are the major structural components of elastic fibers, mutations in elastin and fibrillin-1 lead to narrowing of large arteries in supravascular aortic stenosis and dilation of the ascending aorta in Marfan syndrome, respectively. A genetic approach was therefore used here to distinguish the differential contributions of elastin and fibrillin-1 to arterial development and compliance.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>Methods and Results\u2014<\/jats:italic>\n            <\/jats:bold>\n            Key parameters of cardiovascular function were compared among adult mice haploinsufficient for elastin (\n            <jats:italic>Eln<\/jats:italic>\n            <jats:sup>+\/\u2212<\/jats:sup>\n            ), fibrillin-1 (\n            <jats:italic>Fbn1<\/jats:italic>\n            <jats:sup>+\/\u2212<\/jats:sup>\n            ), or both proteins (\n            <jats:italic>dHet<\/jats:italic>\n            ). Physiological and morphological comparisons correlate elastin haploinsufficiency with increased blood pressure and vessel length and tortuosity in\n            <jats:italic>dHet<\/jats:italic>\n            mice, and fibrillin-1 haploinsufficiency with increased aortic diameter in the same mutant animals. Mechanical tests confirm that elastin and fibrillin-1 impart elastic recoil and tensile strength to the aortic wall, respectively. Additional ex vivo analyses demonstrate additive and overlapping contributions of elastin and fibrillin-1 to the material properties of vascular tissues. Lastly, light and electron microscopy evidence implicates fibrillin-1 in the hypertension-promoted remodeling of the elastin-deficient aorta.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>Conclusions\u2014<\/jats:italic>\n            <\/jats:bold>\n            These results demonstrate that elastin and fibrillin-1 have both differential and complementary roles in arterial wall formation and function, and advance our knowledge of the structural determinants of vascular physiology and disease.\n          <\/jats:p>","DOI":"10.1161\/atvbaha.109.193227","type":"journal-article","created":{"date-parts":[[2009,10,23]],"date-time":"2009-10-23T02:15:59Z","timestamp":1256264159000},"page":"2083-2089","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":80,"title":["Discrete Contributions of Elastic Fiber Components to Arterial Development and Mechanical Compliance"],"prefix":"10.1161","volume":"29","author":[{"given":"Luca","family":"Carta","sequence":"first","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica E.","family":"Wagenseil","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell H.","family":"Knutsen","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boubacar","family":"Mariko","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilles","family":"Faury","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine C.","family":"Davis","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barry","family":"Starcher","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert P.","family":"Mecham","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Ramirez","sequence":"additional","affiliation":[{"name":"From the Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute (L.C., F.R.), Mount Sinai School of Medicine, New York; the Department of Biomedical Engineering (J.E.W.), Saint Louis University, Mo; the Department of Cell Biology and Physiology (R.H.K., R.P.M.), Washington University School of Medicine, St. Louis, Mo; Universite\u0301 Joseph Fourier (B.M., G.F.), Grenoble, France; the Department of Anatomy and Cell Biology (E.C.D.), McGill University, Montreal, Canada; and..."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0070-2153(04)62006-0"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.R900004200"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1050-1738(03)00065-3"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-218"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/30522"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI4487"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.7.3819"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1161\/res.88.1.37"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.21.7.1184"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI19028"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00046.2005"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1089\/rej.2007.0587"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.90563.2008"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1161\/circresaha.108.192054"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511599200"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.3109\/03008209509028401"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/0378-4347(92)80301-6"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M209201200"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1115\/1.3138600"},{"key":"e_1_3_3_20_2","first-page":"89","volume":"68","year":"1993","unstructured":"Davis EC. 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