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Please use this identifier to cite or link to this item: http://hdl.handle.net/123456789/14784

Title: Distribution of BIG-H3 protein in primary and recurrent granular corneal dystrophy
Authors: Saeed Akhtar PhD
Keywords: Granular corneal dystrophy GCD (PGCD); h3 protein; corneal stroma
Issue Date: 2008
Publisher: Acta Ophthalmologica Journal
Abstract: Purpose Granular corneal dystrophy (GCD) is a hereditary disease involving the formation of opaque granular deposits in the corneal stroma. In the present project we investigated the distribution of big-h3 protein in the cornea of primary GCD (PGCD), recurrent GCD (RGCD) and following epithelial debridement of RGCD. Methods The distribution of big-h3 protein was studied in 2 PGCDs, 2 RGCDs, and in epithelial debridement specimens from 2 RCGD corneas. The corneas were fixed in 4% paraformaldehyde at 4°C and embedded in LR white under UV light. The primary antibody big-h3 was located by secondary, goat anti-rabbit antibody conjugated with gold. Results In normal cornea moderate distribution of big-h3 was observed. In PGCD and RGCD corneas, strong labelling for big-h3 was observed in deposits, within very thin microfilaments (nm) and at the inter-lamellar junctions. Labelling was significantly higher in GCD Descemet’s membrane compared to normal Descemet’s membrane. Very strong labelling was observed in the deposits and in the cytoplasm of epithelial cells, of debridement specimens. Conclusion Big-h3 protein, of epithelial origin, is thought to diffuse into the stroma to form stromal deposits. The presence of aggregates of the protein around keratocytes suggests that it is also produced by these cells. The presence of granular deposits and big-h3 protein suggested that the protein migrated from limbal epithelium during resurfacing of host epithelium.
URI: http://hdl.handle.net/123456789/14784
Appears in Collections:College of Applied Medical Sciences

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