{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T10:24:43Z","timestamp":1790936683807,"version":"4.1.0"},"reference-count":75,"publisher":"American Physiological Society","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Cell Physiology"],"published-print":{"date-parts":[[2010,11]]},"abstract":"<jats:p> Microfibrils are macromolecular complexes associated with elastin to form elastic fibers that endow extensible tissues, such as arteries, lungs, and skin, with elasticity property. Fibrillin-1, the main component of microfibrils, is a 350-kDa glycoprotein for which genetic haploinsufficiency in humans can lead to Marfan syndrome, a severe polyfeatured pathology including aortic aneurysms and dissections. Microfibrils and fibrillin-1 fragments mediate adhesion of several cell types, including endothelial cells, while fibrillin-1 additionally triggers lung and mesangial cell migration. However, fibrillin-1-induced intracellular signaling is unknown. We have studied the signaling events induced in human umbilical venous endothelial cells (HUVECs) by aortic microfibrils as well as recombinant fibrillin-1 Arg-Gly-Asp (RGD)-containing fragments PF9 and PF14. Aortic microfibrils and PF14, not PF9, substantially and dose dependently increased HUVEC cytoplasmic and nuclear calcium levels measured using the fluorescent dye Fluo-3. This effect of PF14 was confirmed in bovine aortic endothelial cells. PF14 action in HUVECs was mediated by \u03b1v\u03b23 and \u03b15\u03b21 integrins, phospholipase-C, inosital 1,4,5-trisphosphate, and mobilization of intracellular calcium stores, whereas membrane calcium channels were not or only slightly implicated, as shown in patch-clamp experiments. Finally, PF14 enhanced endothelial cell proliferation and migration. Hence, fibrillin-1 sequences may physiologically activate endothelial cells. Genetic fibrillin-1 deficiency could alter normal endothelial signaling and, since endothelium dysfunction is an important contributor to Marfan syndrome, participate in the arterial anomalies associated with this developmental disease. <\/jats:p>","DOI":"10.1152\/ajpcell.00377.2009","type":"journal-article","created":{"date-parts":[[2010,8,5]],"date-time":"2010-08-05T03:54:33Z","timestamp":1280980473000},"page":"C977-C987","source":"Crossref","is-referenced-by-count":41,"title":["Microfibrils and fibrillin-1 induce integrin-mediated signaling, proliferation and migration in human endothelial cells"],"prefix":"10.1152","volume":"299","author":[{"given":"Boubacar","family":"Mariko","sequence":"first","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zeinab","family":"Ghandour","sequence":"additional","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phanie","family":"Raveaud","sequence":"additional","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micka\u00ebl","family":"Quentin","sequence":"additional","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Usson","sequence":"additional","affiliation":[{"name":"Laboratory TIMC, Team \u201cReconnaissance des Formes et Microscopie Quantitative,\u201d Institut d'Ing\u00e9ni\u00e9rie et de l'Information de Sant\u00e9, Centre National de la Recherche Scientifique, UMR5525, Grenoble; Universit\u00e9 Joseph Fourier, La Tronche, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean","family":"Verdetti","sequence":"additional","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Huber","sequence":"additional","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cay","family":"Kielty","sequence":"additional","affiliation":[{"name":"Wellcome Trust Centre for Cell-Matrix Research, and"},{"name":"Centre for Tissue Regeneration, University of Manchester, Manchester, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilles","family":"Faury","sequence":"additional","affiliation":[{"name":"Laboratoire \u201cPhysiopathologies Vasculaires: Interactions Cellulaires, Signalisation et Vieillissement,\u201d Universit\u00e9 Joseph Fourier, CEA; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U882, Grenoble;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"24","reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2007.060647"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.carrad.2004.08.001"},{"key":"B3","first-page":"231","volume":"64","author":"Balconi G","year":"1986","journal-title":"Med Biol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.152.5.1045"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M303159200"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.003954"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.017525"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1038\/35036035"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1016\/j.cbpa.2005.09.020"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1002\/pmic.200401340"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511599200"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjp.0707181"},{"key":"B13","first-page":"89","volume":"68","author":"Davis EC","year":"1993","journal-title":"Lab Invest"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.107.3.727"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M608204200"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/S0369-8114(01)00147-X"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.82.3.328"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1172\/JCI19028"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.6.2967"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.3109\/08977190109001074"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.16.1.19"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.ehj.2003.08.020"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1007\/s10456-005-9026-5"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.19.12.2871"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M212670200"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1016\/S0070-2153(06)75004-9"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1177\/000331979804900407"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.84.4.995"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1172\/JCI107470"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.0000189559.87328.e4"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.02374"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)67789-6"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.3109\/15419069509081013"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.115.14.2817"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.103.2.445"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M003668200"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65005-5"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M606370200"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V100.13.4495"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1016\/j.str.2004.02.023"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)69279-8"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1172\/JCI4487"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.3109\/03008209809028905"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsb.2006.03.021"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.1161\/hq0701.092136"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-007-0371-x"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2007-0281OC"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.29.17361"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M205630200"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.2174\/157489206775246502"},{"key":"B51","doi-asserted-by":"publisher","DOI":"10.1038\/415171a"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1089\/rej.2007.0587"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(96)00325-0"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ki.5000030"},{"key":"B55","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1995.1177"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.1016\/j.biochi.2004.03.002"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.1590\/S0100-879X2008005000050"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.7.13.8405806"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-003-2297-3"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)98752-1"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.9.4916"},{"key":"B62","doi-asserted-by":"publisher","DOI":"10.1089\/dna.2005.24.218"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200407060"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.1016\/S0070-2153(04)64009-9"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.3109\/03008209709160221"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1994.267.4.C920"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2105-7-520"},{"key":"B68","doi-asserted-by":"publisher","DOI":"10.5551\/jat.11.159"},{"key":"B69","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-005-4522-8"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1016\/j.biomaterials.2007.08.019"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.99.7.909"},{"key":"B72","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvs.2007.09.016"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.1038\/415168a"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.24.2.638-650.2004"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.129.4.1165"}],"container-title":["American Journal of Physiology-Cell Physiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.physiology.org\/doi\/pdf\/10.1152\/ajpcell.00377.2009","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,6,17]],"date-time":"2024-06-17T17:05:09Z","timestamp":1718643909000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.physiology.org\/doi\/10.1152\/ajpcell.00377.2009"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,11]]},"references-count":75,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2010,11]]}},"alternative-id":["10.1152\/ajpcell.00377.2009"],"URL":"https:\/\/doi.org\/10.1152\/ajpcell.00377.2009","relation":{},"ISSN":["0363-6143","1522-1563"],"issn-type":[{"value":"0363-6143","type":"print"},{"value":"1522-1563","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,11]]}}}