Extracellular matrix alterations in late-onset Fuchs' corneal dystrophy

Weller JM, Zenkel M, Schlötzer-Schrehardt U, Bachmann B, Tourtas T, Kruse F (2014)


Publication Type: Journal article

Publication year: 2014

Journal

Publisher: Association for Research in Vision and Ophthalmology (ARVO)

Book Volume: 55

Pages Range: 3700-8

Journal Issue: 6

DOI: 10.1167/iovs.14-14154

Abstract

To characterize the alterations of extracellular matrix proteins in Descemet's membranes (DM) of patients with late-onset Fuchs' corneal dystrophy (FCD) and to differentiate them from nonspecific alterations in pseudophakic bullous keratopathy (PBK).Human DM-endothelial cell complexes were obtained from patients with late-onset FCD (n = 40), PBK (n = 6), and control eyes (n = 5). Gene expression profiles of endothelial cells were compared using a commercial real-time PCR array and quantitative real-time PCR assays for confirmation of differentially expressed genes. A total of 24 extracellular matrix proteins were also localized in cryosections of corneal specimens from FCD (n = 10), PBK (n = 4), and control eyes (n = 5) by immunohistochemistry.Polymerase chain reaction array analysis revealed a significant upregulation of 27 out of 84 extracellular matrix-related genes including collagens, proteoglycans, glycoproteins, cell adhesion molecules, and matrix metalloproteinases in FCD specimens as compared to normal controls, which could be partly confirmed and quantified by real-time PCR. Comparative analysis of FCD and PBK specimens showed a significant and consistent FCD-specific upregulation of collagen types I, III, and XVI; fibronectin; agrin; clusterin; transforming growth factor beta-induced (TGFBI); and integrin ?4 (3- to 18-fold, P < 0.05). Immunohistochemistry revealed an increased labeling of collagen (types III, VII, XV, XVI), agrin, fibulin-2, TGFBI, versican, and clusterin in the DM of FCD specimens compared to PBK specimens.The findings provide evidence for a specific upregulation, production, and deposition of collagen types III and XVI, agrin, TGFBI, and clusterin in late-onset FCD and thus point to the importance of matrix alterations in the pathophysiology of FCD.

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How to cite

APA:

Weller, J.M., Zenkel, M., Schlötzer-Schrehardt, U., Bachmann, B., Tourtas, T., & Kruse, F. (2014). Extracellular matrix alterations in late-onset Fuchs' corneal dystrophy. Investigative Ophthalmology & Visual Science, 55(6), 3700-8. https://doi.org/10.1167/iovs.14-14154

MLA:

Weller, Julia M., et al. "Extracellular matrix alterations in late-onset Fuchs' corneal dystrophy." Investigative Ophthalmology & Visual Science 55.6 (2014): 3700-8.

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