{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T13:43:06Z","timestamp":1790516586720,"version":"4.1.0"},"reference-count":42,"publisher":"National Academy of Sciences","issue":"45","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2015,11,10]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Marfan syndrome (MFS) is a connective tissue disorder caused by mutations of fibrillin-1 (FBN1), the main component of extracellular matrix microfibrils.\n                    <jats:italic>FBN1<\/jats:italic>\n                    mutations predispose to thoracic aortic aneurysm and rupture and are associated with increased TGF\u03b2 signaling. TGF\u03b2 is secreted from cells complexed with latent TGF\u03b2 binding protein (LTBP), a protein that targets TGF\u03b2 to the ECM through interaction with fibrillin-1. One hypothesis proposes that aortic disease in MFS is due to the release of LTBP\/TGF\u03b2 complexes in the aortic wall. We suppressed the expression of\n                    <jats:italic>Ltbp3<\/jats:italic>\n                    in an MFS mouse model and observed essentially no aortic aneurysm and rupture in these compound mice. Our data suggest a key role for LTBP-3 in MFS aortic disease and provide a potential therapeutic point for intervention.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1507652112","type":"journal-article","created":{"date-parts":[[2015,10,22]],"date-time":"2015-10-22T23:13:46Z","timestamp":1445555626000},"page":"14012-14017","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":55,"title":["Genetic analysis of the contribution of LTBP-3 to thoracic aneurysm in Marfan syndrome"],"prefix":"10.1073","volume":"112","author":[{"given":"Lior","family":"Zilberberg","sequence":"first","affiliation":[{"name":"Department of Cell Biology, New York University School of Medicine, New York, NY 10016;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin K. 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