Immunohistochemical Localization of Fibrillin in Human Ocular Tissues: Relevance to the Marfan Syndrome

doi:10.1001/archopht.1995.01100010105028

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Vol. 113 No. 1, January 1995

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Immunohistochemical Localization of Fibrillin in Human Ocular Tissues

Relevance to the Marfan Syndrome

H. Matthew Wheatley, MD; Elias I. Traboulsi, MD; Brian E. Flowers, MD; Irene H. Maumenee, MD; Dmitri Azar, MD; Reed E. Pyeritz, MD; Judith A. Whittum-Hudson, PhD

Arch Ophthalmol. 1995;113(1):103-109.

Abstract

Objective
To better understand the ocular manifestations of the Marfansyndrome, we investigated the distribution of fibrillin in normalhuman ocular tissues. Fibrillin, a microfibrillar glycoproteincomponent of the extracellular matrix, has been found to bethe defective gene product in the Marfan syndrome.

Methods
Frozen sections from seven pairs of normal eyes were stainedwith mouse anti—human fibrillin antibodies using the avidin-biotinimmunoperoxidase technique.

Results
In the anterior segment, the following exhibited positive stainingfor fibrillin: the lens capsule and zonules; connective tissuesof the iris, ciliary body, ciliary processes, and conjunctiva;and the basement membrane regions of the corneal epitheliumand endothelium of Schlemm's canal. Posteriorly, fibrillin localizedto the lamina cribrosa, sclera, choroid, and Bruch's membrane.

Conclusions
Fibrillin is widely distributed in ocular connective tissues.The implications of defects in these tissues and the resultantocular abnormalities in the Marfan syndrome such as ectopialentis and glaucoma are discussed.

Author Affiliations

From The Johns Hopkins Center for Hereditary Eye Diseases (Drs Wheatley, Traboulsi, Flowers, and Maumenee), the Keratorefractive (Dr Azar) and Cornea (Dr Azar) Services, and the Ocular Immunology Laboratory (Dr Whittum-Hudson), Wilmer Ophthalmological Institute, The Johns Hopkins University School of Medicine, Baltimore, Md; and the Department of Human Genetics (Dr Pyeritz), Allegheny-Singer Research Institute, Pittsburgh, Pa.

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