May 2004
Volume 45, Issue 13
ARVO Annual Meeting Abstract  |   May 2004
Keratoepithelin In Secondary Corneal Amyloidosis
Author Affiliations & Notes
  • D. Suesskind
    Department of Ophthalmology, University, Bonn, Germany
  • C. Auw–Haedrich
    Department of Ophthalmology, University, Freiburg, Germany
  • K.U. Loeffler
    Department of Ophthalmology, University, Bonn, Germany
  • D.F. Schorderet
    Division of Medical Genetics, CHUV, Lausanne, Switzerland
  • F.L. Munier
    Division of Medical Genetics, CHUV, Lausanne, Switzerland
  • Footnotes
    Commercial Relationships  D. Suesskind, None; C. Auw–Haedrich, None; K.U. Loeffler, None; D.F. Schorderet, None; F.L. Munier, None.
  • Footnotes
    Support  none
Investigative Ophthalmology & Visual Science May 2004, Vol.45, 4677. doi:
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      D. Suesskind, C. Auw–Haedrich, K.U. Loeffler, D.F. Schorderet, F.L. Munier; Keratoepithelin In Secondary Corneal Amyloidosis . Invest. Ophthalmol. Vis. Sci. 2004;45(13):4677.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract: : Purpose: Amyloid is found in several corneal dystrophies including lattice corneal dystrophies (LCD) type I, IA, IIIA, IV, VI and VII and Avellino corneal dystrophy. Recently, point mutations in the BIGH3 gene encoding for keratoepithelin (KE) have been demonstrated in these corneal disease entities. KE is a 68 kDa protein that is expressed in many tissues including the cornea. The precise function of KE is still unknown but it is supposed to play a role in cell adhesion as a protein of the extracellular matrix (ECM). Amyloid is also found in secondary deposits due to degenerative corneal pannus or different inflammatory ocular diseases. We intended to investigate if KE would also be a component of these 2° amyloid deposits. Methods: Immunohistochemical staining with a polyclonal antibody to KE was performed on formalin–fixed paraffin–embedded tissue of 6 different corneal buttons with 2° amyloid obtained after keratoplasty. The diagnosis of 2° amyloidosis had been established by light microscopy and congo red staining and was due to Fuchs endothelial dystrophy (FED) with bullous keratopathy and/or recurrent erosions in all cases. 2 cases of LCD type I and 4 corneas with keratoconus or FED without amyloid served as positive and comparative controls. Results: All corneas with 2° amyloidosis as well as LCD type I revealed positive staining in the respective amyloid deposits. The intensity of staining was variable among the specimens. As has been described in the literature, Bowman's layer and Descemt's membrane were also labeled in a number of cases independent of the disease. Conclusions: KE is found not only in primary amyloid deposits of hereditary corneal dystrophies but also in 2° amyloidosis of the cornea. KE is supposed to be involved in corneal development and wound healing. It has been shown that expression of KE is increased under those conditions where healing is associated with increased synthesis of new ECM. The corneal buttons investigated were obtained after keratoplasty because of epithelial healing problems and subsequent scarring processes. Perhaps in this environment KE is altered e.g. by proteolysis in such a way that it is able to build fibrils which aggregate to form amyloid deposits.

Keywords: degenerations/dystrophies • immunohistochemistry • pathology: human 

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