{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T09:54:57Z","timestamp":1792662897635,"version":"4.5.0"},"reference-count":54,"publisher":"The Company of Biologists","issue":"1","license":[{"start":{"date-parts":[[2020,9,3]],"date-time":"2020-09-03T00:00:00Z","timestamp":1599091200000},"content-version":"vor","delay-in-days":611,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 EY029130"],"award-info":[{"award-number":["R01 EY029130"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30 EY02687"],"award-info":[{"award-number":["P30 EY02687"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["T32 EY013360"],"award-info":[{"award-number":["T32 EY013360"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002247","name":"National Marfan Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002247","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001818","name":"Research to Prevent Blindness","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001818","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,1,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Fibrillin is an evolutionarily ancient protein that lends elasticity and resiliency to a variety of tissues. In humans, mutations in fibrillin-1 cause Marfan and related syndromes, conditions in which the eye is often severely affected. To gain insights into the ocular sequelae of Marfan syndrome, we targeted Fbn1 in mouse lens or non-pigmented ciliary epithelium (NPCE). Conditional knockout of Fbn1 in NPCE, but not lens, profoundly affected the ciliary zonule, the system of fibrillin-rich fibers that centers the lens in the eye. The tensile strength of the fibrillin-depleted zonule was reduced substantially, due to a shift toward production of smaller caliber fibers. By 3 months, zonular fibers invariably ruptured and mice developed ectopia lentis, a hallmark of Marfan syndrome. At later stages, untethered lenses lost their polarity and developed cataracts, and the length and volume of mutant eyes increased. This model thus captures key aspects of Marfan-related syndromes, providing insights into the role of fibrillin-1 in eye development and disease.<\/jats:p>","DOI":"10.1242\/dmm.037283","type":"journal-article","created":{"date-parts":[[2019,1,14]],"date-time":"2019-01-14T19:15:13Z","timestamp":1547493313000},"source":"Crossref","is-referenced-by-count":61,"title":["Targeted deletion of fibrillin-1 in the mouse eye results in ectopia lentis and other ocular phenotypes associated with Marfan syndrome"],"prefix":"10.1242","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4420-7524","authenticated-orcid":false,"given":"Wendell","family":"Jones","sequence":"first","affiliation":[{"name":"Washington University School of Medicine 1 Department of Ophthalmology and Visual Sciences , , 660 S. Euclid Ave, Box\u00a08096, St. Louis, MO 63117 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5236-5015","authenticated-orcid":false,"given":"Juan","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"St Louis College of Pharmacy 2 , Department of Basic Sciences, 4588 Parkview Place, St. Louis, MO 63110 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8337-2760","authenticated-orcid":false,"given":"Steven","family":"Bassnett","sequence":"additional","affiliation":[{"name":"Washington University School of Medicine 1 Department of Ophthalmology and Visual Sciences , , 660 S. Euclid Ave, Box\u00a08096, St. Louis, MO 63117 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2019,1,25]]},"reference":[{"key":"2024061303491732600_DMM037283C1","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1016\/j.ajhg.2009.01.007","article-title":"A homozygous mutation in ADAMTSL4 causes autosomal-recessive isolated ectopia lentis","volume":"84","author":"Ahram","year":"2009","journal-title":"Am. J. Hum. Genet."},{"key":"2024061303491732600_DMM037283C2","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1083\/jcb.200105046","article-title":"Regulation of limb patterning by extracellular microfibrils","volume":"154","author":"Arteaga-Solis","year":"2001","journal-title":"J. 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