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

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

Research output: Contribution to journalArticle

Abstract

Objective: To better understand the ocular manifestations of the Marfan syndrome, we investigated the distribution of fibrillin in normal human ocular tissues. Fibrillin, a microfibrillar glycoprotein component of the extracellular matrix, has been found to be the defective gene product in the Marfan syndrome. Methods: Frozen sections from seven pairs of normal eyes were stained with mouse anti—human fibrillin antibodies using the avidin-biotin immunoperoxidase technique. Results: In the anterior segment, the following exhibited positive staining for fibrillin: the lens capsule and zonules; connective tissues of the iris, ciliary body, ciliary processes, and conjunctiva; and the basement membrane regions of the corneal epithelium and endothelium of Schlemm's canal. Posteriorly, fibrillin localized to 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 resultant ocular abnormalities in the Marfan syndrome such as ectopia lentis and glaucoma are discussed.

Original languageEnglish (US)
Pages (from-to)103-109
Number of pages7
JournalArchives of Ophthalmology
Volume113
Issue number1
DOIs
StatePublished - 1995

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Marfan Syndrome
Connective Tissue
Eye Manifestations
Ectopia Lentis
Eye Abnormalities
Bruch Membrane
Corneal Endothelium
Corneal Epithelium
Ciliary Body
Sclera
Choroid
Avidin
Conjunctiva
Frozen Sections
Iris
Biotin
Immunoenzyme Techniques
Basement Membrane
Glaucoma
Lenses

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Wheatley, H. M., Traboulsi, E. I., Flowers, B. E., Maumenee, I. H., Azar, D., Pyeritz, R. E., & Whittum Hudson, J. A. (1995). Immunohistochemical Localization of Fibrillin in Human Ocular Tissues: Relevance to the Marfan Syndrome. Archives of Ophthalmology, 113(1), 103-109. https://doi.org/10.1001/archopht.1995.01100010105028

Immunohistochemical Localization of Fibrillin in Human Ocular Tissues : Relevance to the Marfan Syndrome. / Wheatley, H. Matthew; Traboulsi, Elias I.; Flowers, Brian E.; Maumenee, Irene H.; Azar, Dmitri; Pyeritz, Reed E.; Whittum Hudson, Judith A.

In: Archives of Ophthalmology, Vol. 113, No. 1, 1995, p. 103-109.

Research output: Contribution to journalArticle

Wheatley, HM, Traboulsi, EI, Flowers, BE, Maumenee, IH, Azar, D, Pyeritz, RE & Whittum Hudson, JA 1995, 'Immunohistochemical Localization of Fibrillin in Human Ocular Tissues: Relevance to the Marfan Syndrome', Archives of Ophthalmology, vol. 113, no. 1, pp. 103-109. https://doi.org/10.1001/archopht.1995.01100010105028
Wheatley, H. Matthew ; Traboulsi, Elias I. ; Flowers, Brian E. ; Maumenee, Irene H. ; Azar, Dmitri ; Pyeritz, Reed E. ; Whittum Hudson, Judith A. / Immunohistochemical Localization of Fibrillin in Human Ocular Tissues : Relevance to the Marfan Syndrome. In: Archives of Ophthalmology. 1995 ; Vol. 113, No. 1. pp. 103-109.
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