| Autor: |
Rodrigo Barbosa de, Souza, Isabela Gerdes, Gyuricza, Luara Lucena, Cassiano, Luis Ernesto, Farinha-Arcieri, Ana Maria, Alvim Liberatore, Sheila, Schuindt do Carmo, Waldir, Caldeira, Marcio V, Cruz, Alberto F, Ribeiro, Roberto Carlos, Tedesco, Dieter P, Reinhardt, Ricardo, Smith, Ivan Hong, Jun Koh, Lygia V, Pereira |
| Rok vydání: |
2020 |
| Předmět: |
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| Zdroj: |
Experimental eye research. 204 |
| ISSN: |
1096-0007 |
| Popis: |
Fibrillin-1 and -2 are major components of tissue microfibrils that compose the ciliary zonule and cornea. While mutations in human fibrillin-1 lead to ectopia lentis, a major manifestation of Marfan syndrome (MFS), in mice fibrillin-2 can compensate for reduced/lack of fibrillin-1 and maintain the integrity of ocular structures. Here we examine the consequences of a heterozygous dominant-negative mutation in the Fbn1 gene in the ocular system of the mgΔEyes from mgΔMutant mice presented a significantly larger distance of the ciliary body to the lens at 3 and 6 months of age when compared to wild-type, and ectopia lentis. Immunofluorescence and SEM corroborated those findings in MFS mice, revealing a disorganized mesh of microfibrils on the floor of the ciliary body. Moreover, mutant mice also had a larger volume of the anterior chamber, possibly due to excess aqueous humor. Finally, losartan treatment had limited efficacy in improving ocular phenotypes.In contrast with null or hypomorphic mutations, expression of a dominant-negative form of fibrillin-1 leads to disruption of microfibrils in the zonule of mice. This in turn causes lens dislocation and enlargement of the anterior chamber. Therefore, heterozygous mgΔ |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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