{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T08:38:19Z","timestamp":1792226299072,"version":"4.4.0"},"reference-count":16,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2012,3,31]],"date-time":"2012-03-31T00:00:00Z","timestamp":1333152000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genesis"],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Loss\u2010of\u2010function experiments in mice have yielded invaluable mechanistic insights into the pathogenesis of Marfan syndrome (MFS) and implicitly, into the multiple roles fibrillin\u20101 microfibrils play in the developing and adult organism. Unfortunately, neonatal death from aortic complications of mice lacking fibrillin\u20101 (<jats:italic>Fbn1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice) has limited the scope of these studies. Here, we report the creation of a conditional mutant allele (<jats:italic>Fbn1<\/jats:italic><jats:sup><jats:italic>fneo<\/jats:italic><\/jats:sup>) that contains loxP sites bordering exon1 of <jats:italic>Fbn1<\/jats:italic> and an frt\u2010flanked <jats:italic>neo<\/jats:italic> expression cassette downstream of it. <jats:italic>Fbn1<\/jats:italic><jats:sup><jats:italic>fneo<\/jats:italic>\/+<\/jats:sup> mice were crossed with <jats:italic>FLPeR<\/jats:italic> mice and the resulting <jats:italic>Fbn1<\/jats:italic><jats:sup><jats:italic>Lox<\/jats:italic>\/+<\/jats:sup> progeny were crossed with <jats:italic>Fbn1<\/jats:italic><jats:sup>+\/\u2212<\/jats:sup>;<jats:italic>CMV\u2010Cre<\/jats:italic> mice to generate <jats:italic>Fbn1<\/jats:italic><jats:sup><jats:italic>CMV<\/jats:italic>\u2212\/\u2212<\/jats:sup> mice, which were found to phenocopy the vascular abnormalities of <jats:italic>Fbn1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice. Furthermore, mating <jats:italic>Fbn1<\/jats:italic><jats:sup><jats:italic>Lox<\/jats:italic>\/+<\/jats:sup> mice with <jats:italic>Prx1\u2010Cre<\/jats:italic> or <jats:italic>Osx\u2010Cre<\/jats:italic> mice revealed an unappreciated role of fibrillin\u20101 microfibrils in restricting osteoprogenitor cell recruitment. <jats:italic>Fbn1<\/jats:italic><jats:sup><jats:italic>Lox<\/jats:italic>\/+<\/jats:sup> mice are, therefore, an informative genetic resource to further dissect MFS pathogenesis and the role of extracellular fibrillin\u20101 assemblies in organ development and homeostasis. genesis 50:635\u2013641, 2012. \u00a9 2012 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/dvg.22022","type":"journal-article","created":{"date-parts":[[2012,2,29]],"date-time":"2012-02-29T04:35:00Z","timestamp":1330490100000},"page":"635-641","source":"Crossref","is-referenced-by-count":25,"title":["Generation of <i>Fbn1<\/i> conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment"],"prefix":"10.1002","volume":"50","author":[{"given":"Jason R.","family":"Cook","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Smaldone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmine","family":"Cozzolino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"del Solar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sui","family":"Lee\u2010Arteaga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harikiran","family":"Nistala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Ramirez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,3,31]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511599200"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/1526-968X(200011\/12)28:3\/4<106::AID-GENE30>3.0.CO;2-T"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1124287"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1192149"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002239900121"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0014136"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/gene.10092"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq409"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201003089"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.125328"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-218"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.7.3819"},{"key":"e_1_2_4_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gde.2007.04.006"},{"key":"e_1_2_4_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ceb.2009.05.005"},{"key":"e_1_2_4_16_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.02480"},{"key":"e_1_2_4_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/23.24.5080"}],"container-title":["genesis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fdvg.22022","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/dvg.22022","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,21]],"date-time":"2023-11-21T07:24:49Z","timestamp":1700551489000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/dvg.22022"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,3,31]]},"references-count":16,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2012,8]]}},"alternative-id":["10.1002\/dvg.22022"],"URL":"https:\/\/doi.org\/10.1002\/dvg.22022","archive":["Portico"],"relation":{},"ISSN":["1526-954X","1526-968X"],"issn-type":[{"value":"1526-954X","type":"print"},{"value":"1526-968X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,3,31]]}}}