ArticleInvestigative ophthalmology & visual science2013
Nonselective assembly of fibrillin 1 and fibrillin 2 in the rodent ocular zonule and in cultured cells: implications for Marfan syndrome.
Article in Investigative ophthalmology & visual science, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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34 citing papers in PubMed, 53 citations in OpenAlex.
- Gene polymorphisms associated with corneal curvature, astigmatism and its vector components in children.Eye and vision (London, England) · 2025Article
- Exploring thoracic aorta ECM alterations in Marfan syndrome: insights into aorta wall structure.Scientific reports · 2025Article
- Unanswered questions regarding the pathogenesis of late onset posterior capsular opacification.Frontiers in ophthalmology · 2025Review
- Extracellular matrix protein composition dynamically changes during murine forelimb development.iScience · 2024Article
- Marfan syndrome in a Ghanaian male: The diagnostic challenges.Clinical case reports · 2024Article
- Genetic mutation and aqueous humor metabolites alterations in a family with Marfan syndrome.Heliyon · 2024Article
- Genetic models of fibrillinopathies.Genetics · 2024Review
- Marfan syndrome: insights from animal models.Frontiers in genetics · 2024Review
- Screening and expression validation of key proteins for secondary hair follicle growth in cashmere goats based on iTRAQ quantitative proteomics technology.Frontiers in veterinary science · 2024Article
- The fibrillinopathies: New insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2.Human mutation · 2022Review
- Article
- Marfan syndrome.Nature reviews. Disease primers · 2021Review
- Altered Ocular Fibrillin Microfibril Composition in Mice With a Glaucoma-Causing Mutation of Adamts10.Investigative ophthalmology & visual science · 2021Article
- Zinn's zonule.Progress in retinal and eye research · 2021Review
- Nanophthalmos-Associated MYRF Gene Mutation Causes Ciliary Zonule Defects in Mice.Investigative ophthalmology & visual science · 2021Article
- Dynamics of the lens basement membrane capsule and its interaction with connective tissue-like extracapsular matrix proteins.Matrix biology : journal of the International Society for Matrix Biology · 2021Article
- Latent-transforming growth factor beta-binding protein-2 (LTBP-2) is required for longevity but not for development of zonular fibers.Matrix biology : journal of the International Society for Matrix Biology · 2021Article
- An immune response to the avascular lens following wounding of the cornea involves ciliary zonule fibrils.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2020Article
- Cell-Based Interaction Analysis of ADAMTS Proteases and ADAMTS-Like Proteins with Fibrillin Microfibrils.Methods in molecular biology (Clifton, N.J.) · 2020Article
- Limb- and tendon-specific Adamtsl2 deletion identifies a role for ADAMTSL2 in tendon growth in a mouse model for geleophysic dysplasia.Matrix biology : journal of the International Society for Matrix Biology · 2019Article
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Authors and funding
10 authors at 6 institutions in 2 countries.
Funding
Abstract
purposeFibrillins are the major constituent of tissue microfibrils, which form the ocular zonule. In Marfan syndrome (MFS), FBN1 mutations lead to ectopia lentis. The goal of this work was to investigate zonule composition and formation in fibrillin-deficient and wild-type mice.
methodsImmunofluorescence staining of eyes from wild-type, Fbn1-deficient, and Fbn2-deficient mice, as well as other species, was performed using monospecific fibrillin 1 and fibrillin 2 antibodies. The zonule of Fbn1-deficient and Fbn2-deficient mice was studied by electron microscopy. Microfibril formation in vitro was evaluated by immunofluorescence microscopy of cultured nonpigmented ciliary epithelial cells and fibroblasts.
resultsA zonule was present in both Fbn1-deficient and Fbn2-deficient mouse eyes. Immunofluorescence demonstrated that the zonule of Fbn1-deficient mice, wild-type mice, rats, and hamsters contained fibrillin 2. The zonule of Fbn2(-/-) mice contained fibrillin 1. Fibrillin 1 and fibrillin 2 colocalized in microfibrils formed in human nonpigmented ciliary epithelium cultures. Like fibrillin 1, fibrillin 2 microfibril assembly was fibronectin dependent and initiated by cell surface punctate deposits that elongated to form microfibrils.
conclusionsThese data suggest that fibrillin 1 assembly and fibrillin 2 assembly share similar mechanisms. Microfibril composition depends substantially on the local levels of fibrillin isoforms and is not highly selective in regard to the isoform. This raises the intriguing possibility that the zonule could be strengthened in MFS by inducing fibrillin 2 expression in ciliary epithelium. The presence of fibrillin 2 in the murine zonule and an intact zonule in Fbn1-knockout mice may limit the utility of rodent models for studying ectopia lentis in MFS.
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