{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T21:10:05Z","timestamp":1789679405861,"version":"4.0.1"},"reference-count":81,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2024,12,18]],"date-time":"2024-12-18T00:00:00Z","timestamp":1734480000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIH","award":["R01EY029130"],"award-info":[{"award-number":["R01EY029130"]}]},{"name":"NIH","award":["R01EY020894"],"award-info":[{"award-number":["R01EY020894"]}]},{"name":"NIH","award":["P30 EY002687"],"award-info":[{"award-number":["P30 EY002687"]}]},{"name":"Department of Ophthalmology and Visual Sciences from Research to Prevent Blindness","award":["R01EY029130"],"award-info":[{"award-number":["R01EY029130"]}]},{"name":"Department of Ophthalmology and Visual Sciences from Research to Prevent Blindness","award":["R01EY020894"],"award-info":[{"award-number":["R01EY020894"]}]},{"name":"Department of Ophthalmology and Visual Sciences from Research to Prevent Blindness","award":["P30 EY002687"],"award-info":[{"award-number":["P30 EY002687"]}]},{"name":"Ines Mandl Research Foundation","award":["R01EY029130"],"award-info":[{"award-number":["R01EY029130"]}]},{"name":"Ines Mandl Research Foundation","award":["R01EY020894"],"award-info":[{"award-number":["R01EY020894"]}]},{"name":"Ines Mandl Research Foundation","award":["P30 EY002687"],"award-info":[{"award-number":["P30 EY002687"]}]},{"name":"Grace Nelson Lacy Fund","award":["R01EY029130"],"award-info":[{"award-number":["R01EY029130"]}]},{"name":"Grace Nelson Lacy Fund","award":["R01EY020894"],"award-info":[{"award-number":["R01EY020894"]}]},{"name":"Grace Nelson Lacy Fund","award":["P30 EY002687"],"award-info":[{"award-number":["P30 EY002687"]}]},{"name":"Glaucoma Foundation","award":["R01EY029130"],"award-info":[{"award-number":["R01EY029130"]}]},{"name":"Glaucoma Foundation","award":["R01EY020894"],"award-info":[{"award-number":["R01EY020894"]}]},{"name":"Glaucoma Foundation","award":["P30 EY002687"],"award-info":[{"award-number":["P30 EY002687"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Marfan syndrome is an inherited connective tissue disorder that affects the cardiovascular, musculoskeletal, and ocular systems. It is caused by pathogenic variants in the fibrillin-1 gene (FBN1). Fibrillin is a primary component of microfibrils, which are found throughout the extracellular matrix (ECM) and provide elasticity and resilience to connective tissue. Microfibrils also play a role in signaling by sequestering growth factors and interacting with cell surface receptors. In many tissues, microfibrils are interwoven with elastin, collagens, and other elements of the ECM. However, uniquely in the ciliary zonule of the eye, microfibrils exist in cell-free bundles largely devoid of other components. This structure offers a rare opportunity to study a pure population of fibrillin microfibrils in a relatively native state. Here, we briefly review the organization of the zonule and describe recent experiments in which we measure zonular biomechanics, providing insights into microfibril dynamics that would be challenging to obtain in other contexts.<\/jats:p>","DOI":"10.3390\/cells13242097","type":"journal-article","created":{"date-parts":[[2024,12,18]],"date-time":"2024-12-18T08:56:32Z","timestamp":1734512192000},"page":"2097","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule"],"prefix":"10.3390","volume":"13","author":[{"given":"Pooja","family":"Rathaur","sequence":"first","affiliation":[{"name":"Department of Ophthalmology & Visual Sciences, Washington University School of Medicine, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6360-0770","authenticated-orcid":false,"given":"Juan","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Department of Basic Sciences, University of Health Sciences and Pharmacy in St. Louis, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5226-8648","authenticated-orcid":false,"given":"John","family":"Kuchtey","sequence":"additional","affiliation":[{"name":"Vanderbilt Eye Institute, Department of Ophthalmology & Visual Sciences, Vanderbilt University Medical Center, Nashville, TN 37232, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-6778-3329","authenticated-orcid":false,"given":"Samuel","family":"Insignares","sequence":"additional","affiliation":[{"name":"Vanderbilt Eye Institute, Department of Ophthalmology & Visual Sciences, Vanderbilt University Medical Center, Nashville, TN 37232, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendell B.","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology & Visual Sciences, Washington University School of Medicine, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0353-3315","authenticated-orcid":false,"given":"Rachel W.","family":"Kuchtey","sequence":"additional","affiliation":[{"name":"Vanderbilt Eye Institute, Department of Ophthalmology & Visual Sciences, Vanderbilt University Medical Center, Nashville, TN 37232, USA"},{"name":"Department of Molecular Physiology and Biophysics, Vanderbilt University Medical Center, Nashville, TN 37232, 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":"Department of Ophthalmology & Visual Sciences, Washington University School of Medicine, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,12,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1038\/352337a0","article-title":"Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene","volume":"352","author":"Dietz","year":"1991","journal-title":"Nature"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1016\/S0140-6736(05)67789-6","article-title":"Marfan\u2019s syndrome","volume":"366","author":"Judge","year":"2005","journal-title":"Lancet"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1038\/s41572-021-00298-7","article-title":"Marfan syndrome","volume":"7","author":"Milewicz","year":"2021","journal-title":"Nat. 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