ArticleInvestigative ophthalmology & visual science2017
The Structural Role of Elastic Fibers in the Cornea Investigated Using a Mouse Model for Marfan Syndrome.
Article in Investigative ophthalmology & visual science, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 1 of them a synthesis that pooled it.
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Who cites it
26 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- Cross-ancestry genome-wide association analysis of corneal thickness strengthens link between complex and Mendelian eye diseases.Nature communications · 2018Pooled it
- Novel findings of corneal neural features in FBN1-related marfan syndrome.International ophthalmology · 2026Article
- Degradation of Elastic Fiber Triggers Lacrimal Gland Dysfunction in Marfan Syndrome Mice.Investigative ophthalmology & visual science · 2025Article
- Gene polymorphisms associated with corneal curvature, astigmatism and its vector components in children.Eye and vision (London, England) · 2025Article
- Structural control of corneal transparency, refractive power and dynamics.Eye (London, England) · 2025Review
- In Search of Mouse Models for Exfoliation Syndrome.American journal of ophthalmology · 2024Article
- A discrete-to-continuum model for the human cornea with application to keratoconus.Royal Society open science · 2024Article
- Genetic mutation and aqueous humor metabolites alterations in a family with Marfan syndrome.Heliyon · 2024Article
- Posterior corneoscleral limbus: Architecture, stem cells, and clinical implications.Progress in retinal and eye research · 2023Review
- Role of elastic fiber degradation in disease pathogenesis.Biochimica et biophysica acta. Molecular basis of disease · 2023Review
- Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome.PloS one · 2023Article
- Elastin turnover in ocular diseases: A special focus on age-related macular degeneration.Experimental eye research · 2022Review
- Structural studies of elastic fibre and microfibrillar proteins.Matrix biology plus · 2021Article
- Scheimpflug-Based Analysis of the Reflectivity of the Cornea in Marfan Syndrome.Translational vision science & technology · 2021Article
- Characteristics of Corneal Higher-Order Aberrations in Congenital Ectopia Lentis Patients.Translational vision science & technology · 2021Article
- Composition, structure and function of the corneal stroma.Experimental eye research · 2020Review
- 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
- Identification of a Primary Stroma and Novel Endothelial Cell Projections in the Developing Human Cornea.Investigative ophthalmology & visual science · 2020Article
- Developmental abnormalities in the cornea of a mouse model for Marfan syndrome.Experimental eye research · 2020Article
- Keratoglobus with ARCL1B (EFEMP2 gene) cutis laxa.American journal of ophthalmology case reports · 2019Article
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Authors and funding
9 authors at 2 institutions in 2 countries.
Funding
Abstract
Purpose: The presence of fibrillin-rich elastic fibers in the cornea has been overlooked in recent years. The aim of the current study was to elucidate their functional role using a mouse model for Marfan syndrome, defective in fibrillin-1, the major structural component of the microfibril bundles that constitute most of the elastic fibers. Methods: Mouse corneas were obtained from animals with a heterozygous fibrillin-1 mutation (Fbn1+/-) and compared to wild type controls. Corneal thickness and radius of curvature were calculated using optical coherence tomography microscopy. Elastic microfibril bundles were quantified and visualized in three-dimensions using serial block face scanning electron microscopy. Transmission electron microscopy was used to analyze stromal ultrastructure and proteoglycan distribution. Center-to-center average interfibrillar spacing was determined using x-ray scattering. Results: Fbn1+/- corneas were significantly thinner than wild types and displayed a higher radius of curvature. In the Fbn1+/- corneas, elastic microfibril bundles were significantly reduced in density and disorganized compared to wild-type controls, in addition to containing a higher average center-to-center collagen interfibrillar spacing in the center of the cornea. No other differences were detected in stromal ultrastructure or proteoglycan distribution between the two groups. Proteoglycan side chains appeared to colocalize with the microfibril bundles. Conclusions: Elastic fibers have an important, multifunctional role in the cornea as highlighted by the differences observed between Fbn1+/- and wild type animals. We contend that the presence of normal quantities of structurally organized elastic fibers are required to maintain the correct geometry of the cornea, which is disrupted in Marfan syndrome.
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