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Role of Decorin Core Protein in Collagen Organisation in Congenital Stromal Corneal Dystrophy (CSCD)

Kamma-Lorger, Christina S, Pinali, Christian, Martínez, Juan Carlos, Harris, John, Young, Robert, Bredrup, Cecile, Crosas, Eva, Malfois, Marc, Rødahl, Eyvind, Meek, Keith and Knupp, Carlo 2016. Role of Decorin Core Protein in Collagen Organisation in Congenital Stromal Corneal Dystrophy (CSCD). PLoS ONE 11 (2) , e0147948. 10.1371/journal.pone.0147948

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Abstract

The role of Decorin in organising the extracellular matrix was examined in normal human corneas and in corneas from patients with Congenital Stromal Corneal Dystrophy (CSCD). In CSCD, corneal clouding occurs due to a truncating mutation (c.967delT) in the decorin (DCN) gene. Normal human Decorin protein and the truncated one were reconstructed in silico using homology modelling techniques to explore structural changes in the diseased protein. Corneal CSCD specimens were also examined using 3-D electron tomography and Small Angle X-ray diffraction (SAXS), to image the collagen-proteoglycan arrangement and to quantify fibrillar diameters, respectively. Homology modelling showed that truncated Decorin had a different spatial geometry to the normal one, with the truncation removing a major part of the site that interacts with collagen, compromising its ability to bind effectively. Electron tomography showed regions of abnormal stroma, where collagen fibrils came together to form thicker fibrillar structures, showing that Decorin plays a key role in the maintenance of the order in the normal corneal extracellular matrix. Average diameter of individual fibrils throughout the thickness of the cornea however remained normal.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Optometry and Vision Sciences
Publisher: Public Library of Science
ISSN: 1932-6203
Funders: MRC
Date of First Compliant Deposit: 8 November 2016
Date of Acceptance: 11 January 2016
Last Modified: 14 May 2019 12:34
URI: http://orca.cf.ac.uk/id/eprint/95909

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