Research progress of fibrillin-1 in ophthalmic diseases
10.3980/j.issn.1672-5123.2026.4.15
- VernacularTitle:原纤维蛋白1在眼科相关疾病中的研究进展
- Author:
Longjiao SUN
1
;
Wenjun JIANG
1
;
Ying WEN
1
Author Information
1. Shandong University of Traditional Chinese Medicine, Jinan 250014,Shandong Province, China
- Publication Type:Journal Article
- Keywords:
fibrillin-1;
lens ectopia;
glaucoma;
applanation
- From:
International Eye Science
2026;26(4):641-645
- CountryChina
- Language:Chinese
-
Abstract:
Fibrillin-1(FBN1)is a large-sized, cysteine-rich, calcium-binding extracellular matrix glycoprotein encoded by the FBN1 gene. As a structural component of microfibrils, this protein is widely distributed in the connective tissues of various ocular structures, including the zonules of Zinn, trabecular meshwork, cornea, retina, and its microvessels, providing mechanical support. Studies have shown that mutations in the FBN1 gene, resulting in decreased protein expression, significantly disrupt microfibril assembly and TGF-β-mediated signaling pathways. These pathogenic mechanisms are strongly linked to the development of key ocular disorders, such as lens dislocation, glaucoma, keratoconus, and retinal vascular diseases. This review summarizes and discusses the clinical manifestations and molecular mechanisms of FBN1-related ophthalmic conditions, with the aim of elucidating their pathogenesis and therapeutic strategies while laying molecular foundations for the development of novel targeted therapies.
