September 1994
Volume 35, Issue 10
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Articles  |   September 1994
Ocular amyloid deposition in familial amyloidosis, Finnish: an analysis of native and variant gelsolin in Meretoja's syndrome.
Author Affiliations
  • T Kivelä
    Department of Ophthalmology, Helsinki University Central Hospital, Finland.
  • A Tarkkanen
    Department of Ophthalmology, Helsinki University Central Hospital, Finland.
  • B Frangione
    Department of Ophthalmology, Helsinki University Central Hospital, Finland.
  • J Ghiso
    Department of Ophthalmology, Helsinki University Central Hospital, Finland.
  • M Haltia
    Department of Ophthalmology, Helsinki University Central Hospital, Finland.
Investigative Ophthalmology & Visual Science September 1994, Vol.35, 3759-3769. doi:
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      T Kivelä, A Tarkkanen, B Frangione, J Ghiso, M Haltia; Ocular amyloid deposition in familial amyloidosis, Finnish: an analysis of native and variant gelsolin in Meretoja's syndrome.. Invest. Ophthalmol. Vis. Sci. 1994;35(10):3759-3769.

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

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Abstract

PURPOSE: To analyze the deposition of amyloid and its precursors in eyes of patients with familial amyloidosis, Finnish (FAF; Meretoja's syndrome), a hereditary systemic amyloidosis. METHODS: Autopsy eyes from three patients with FAF and ten control eyes were studied by Congo red staining and with antibodies to the nonmutated part of gelsolin (GS-2C4), the mutated gelsolin Asn-187 fragment (AGel), and amyloid-P component (AP). RESULTS: Congo red and antisera to AP and AGel bound to amyloid deposits in the cornea and conjunctiva, the sclera, the perineurium of ciliary nerves, the walls of ciliary vessels, the optic nerve sheaths, the stroma of the ciliary body, and along the choriocapillaris. mAb GS-2C4 bound weakly and focally to most deposits and strongly around the choriocapillaris. It labeled the corneal epithelium and endothelium, keratocytes, scleral fibroblasts, trabecular and lens epithelial cells, the ciliary muscle and epithelium, the iris sphincter and dilator, and stromal cells of the conjunctiva and uveal tract. CONCLUSIONS: Local production, especially in the cornea, conjunctiva, sclera, and ciliary muscle, and systemic deposition, particularly in blood vessles and in the sclera, may contribute to amyloid deposits in FAF. To explain the complex pattern of deposition, microenvironmental factors such as lamellar architecture of the cornea and sclera, altered processing of gelsolin, or blood-tissue barriers must be invoked. In addition to corneal lattice dystrophy type II, the observed deposits help to explain glaucoma in patients with FAF.

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