Extracellular matrix alterations in late-onset Fuchs' corneal dystrophy

Area of Science:

• Ophthalmology
• Molecular Biology
• Biochemistry

Background:

• Fuchs' corneal dystrophy (FCD) is a condition affecting the cornea.
• Extracellular matrix (ECM) protein alterations may play a role in FCD.
• Differentiating FCD from pseudophakic bullous keratopathy (PBK) is important.

Purpose of the Study:

• To characterize ECM protein changes in Descemet's membrane (DM) in late-onset FCD.
• To distinguish these FCD-specific alterations from those in PBK.

Main Methods:

• Gene expression profiling of corneal endothelial cells from FCD, PBK, and control eyes using real-time PCR.
• Immunohistochemical localization of 24 ECM proteins in corneal specimens.

Main Results:

• Significant upregulation of 27 ECM-related genes in FCD compared to controls.
• FCD-specific upregulation of collagen types I, III, and XVI, fibronectin, agrin, clusterin, TGFBI, and integrin α4.
• Increased labeling of collagen types III, VII, XV, XVI, agrin, fibulin-2, TGFBI, versican, and clusterin in FCD DM.

Conclusions:

• Evidence supports specific upregulation and deposition of collagen types III and XVI, agrin, TGFBI, and clusterin in late-onset FCD.
• Matrix alterations are implicated in the pathophysiology of FCD.