Increased extracellular deposition of fibrillin-containing fibrils in pseudoexfoliation syndrome

Ursula Schlötzer-Schrehardt, Klaus Von der Mark, Lynn Sakai, Gottfried O H Naumann

Research output: Contribution to journalArticle

108 Citations (Scopus)

Abstract

Purpose. To localize the distribution of fibrillin-containing microfibrils in normal human anterior segment tissues and to characterize the role of fibrillin in the pathogenesis of pseudoexfoliation syndrome. Methods. Anterior segment tissues were obtained from 10 eyes with pseudoexfoliation syndrome and 10 normal eyes and investigated by indirect immunofluorescence and electron microscopic immunogold labeling using a monoclonal antibody to fibrillin-1. Results. In addition to labeling of zonular fibers, fibrillin-immunoreactive microfibrillar bundles generally were found in the corneal stroma; the stromal connective tissues of conjunctiva, ciliary body, and iris, especially in the iris root area; the periphery of Schlemm's canal, the scleral spur, and the most anterior portion of the trabecular meshwork; the ciliary muscle, and the dilator and sphincter muscles of the iris; the basement membranes of peripheral corneal epithelium, conjunctival epithelium, ciliary pigmented epithelium, and the lens capsule. The microfibrillar bundles were found to be isolated or in association with elastic fibers and cellular basement membranes. In pseudoexfoliation eyes, an additional strong immunoreaction was localized to pseudoexfoliation fibers and their microfibrillar subunits in close proximity to surfaces of cells involved in pseudoexfoliation fiber production. Conclusions. The fibrillin-containing microfibrillar system in normal ocular tissues is suggested to have a substantial role in the maintenance of tissue integrity by providing tensile strength and flexibility to mechanically strained tissues. The findings further provide evidence for fibrillin as an intrinsic component of pseudoexfoliation fibers, suggesting the possibility that enhanced expression of fibrillin or abnormal aggregation of fibrillin-containing microfibrils may be involved in the pathogenesis of pseudoexfoliation syndrome.

Original languageEnglish (US)
Pages (from-to)970-984
Number of pages15
JournalInvestigative Ophthalmology and Visual Science
Volume38
Issue number5
StatePublished - May 17 1997
Externally publishedYes

Fingerprint

Exfoliation Syndrome
Iris
Microfibrils
Basement Membrane
Epithelium
Corneal Stroma
Trabecular Meshwork
Muscles
Corneal Epithelium
Ciliary Body
Elastic Tissue
Tensile Strength
Conjunctiva
Indirect Fluorescent Antibody Technique
Fibrillins
Connective Tissue
Lenses
Capsules
Monoclonal Antibodies
Maintenance

Keywords

  • electron microscopy
  • fibrillin
  • immunocytochemistry
  • microfibrils
  • pseudoexfoliation syndrome

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Increased extracellular deposition of fibrillin-containing fibrils in pseudoexfoliation syndrome. / Schlötzer-Schrehardt, Ursula; Von der Mark, Klaus; Sakai, Lynn; Naumann, Gottfried O H.

In: Investigative Ophthalmology and Visual Science, Vol. 38, No. 5, 17.05.1997, p. 970-984.

Research output: Contribution to journalArticle

Schlötzer-Schrehardt, Ursula ; Von der Mark, Klaus ; Sakai, Lynn ; Naumann, Gottfried O H. / Increased extracellular deposition of fibrillin-containing fibrils in pseudoexfoliation syndrome. In: Investigative Ophthalmology and Visual Science. 1997 ; Vol. 38, No. 5. pp. 970-984.
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AU - Sakai, Lynn

AU - Naumann, Gottfried O H

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N2 - Purpose. To localize the distribution of fibrillin-containing microfibrils in normal human anterior segment tissues and to characterize the role of fibrillin in the pathogenesis of pseudoexfoliation syndrome. Methods. Anterior segment tissues were obtained from 10 eyes with pseudoexfoliation syndrome and 10 normal eyes and investigated by indirect immunofluorescence and electron microscopic immunogold labeling using a monoclonal antibody to fibrillin-1. Results. In addition to labeling of zonular fibers, fibrillin-immunoreactive microfibrillar bundles generally were found in the corneal stroma; the stromal connective tissues of conjunctiva, ciliary body, and iris, especially in the iris root area; the periphery of Schlemm's canal, the scleral spur, and the most anterior portion of the trabecular meshwork; the ciliary muscle, and the dilator and sphincter muscles of the iris; the basement membranes of peripheral corneal epithelium, conjunctival epithelium, ciliary pigmented epithelium, and the lens capsule. The microfibrillar bundles were found to be isolated or in association with elastic fibers and cellular basement membranes. In pseudoexfoliation eyes, an additional strong immunoreaction was localized to pseudoexfoliation fibers and their microfibrillar subunits in close proximity to surfaces of cells involved in pseudoexfoliation fiber production. Conclusions. The fibrillin-containing microfibrillar system in normal ocular tissues is suggested to have a substantial role in the maintenance of tissue integrity by providing tensile strength and flexibility to mechanically strained tissues. The findings further provide evidence for fibrillin as an intrinsic component of pseudoexfoliation fibers, suggesting the possibility that enhanced expression of fibrillin or abnormal aggregation of fibrillin-containing microfibrils may be involved in the pathogenesis of pseudoexfoliation syndrome.

AB - Purpose. To localize the distribution of fibrillin-containing microfibrils in normal human anterior segment tissues and to characterize the role of fibrillin in the pathogenesis of pseudoexfoliation syndrome. Methods. Anterior segment tissues were obtained from 10 eyes with pseudoexfoliation syndrome and 10 normal eyes and investigated by indirect immunofluorescence and electron microscopic immunogold labeling using a monoclonal antibody to fibrillin-1. Results. In addition to labeling of zonular fibers, fibrillin-immunoreactive microfibrillar bundles generally were found in the corneal stroma; the stromal connective tissues of conjunctiva, ciliary body, and iris, especially in the iris root area; the periphery of Schlemm's canal, the scleral spur, and the most anterior portion of the trabecular meshwork; the ciliary muscle, and the dilator and sphincter muscles of the iris; the basement membranes of peripheral corneal epithelium, conjunctival epithelium, ciliary pigmented epithelium, and the lens capsule. The microfibrillar bundles were found to be isolated or in association with elastic fibers and cellular basement membranes. In pseudoexfoliation eyes, an additional strong immunoreaction was localized to pseudoexfoliation fibers and their microfibrillar subunits in close proximity to surfaces of cells involved in pseudoexfoliation fiber production. Conclusions. The fibrillin-containing microfibrillar system in normal ocular tissues is suggested to have a substantial role in the maintenance of tissue integrity by providing tensile strength and flexibility to mechanically strained tissues. The findings further provide evidence for fibrillin as an intrinsic component of pseudoexfoliation fibers, suggesting the possibility that enhanced expression of fibrillin or abnormal aggregation of fibrillin-containing microfibrils may be involved in the pathogenesis of pseudoexfoliation syndrome.

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