Immunohistochemical evaluation of conjunctival fibrillin-1 in Marfan syndrome

Anuradha Ganesh, Charles Smith, Wilson Chan, Sheila Unger, Nada Quercia, Maurice Godfrey, Stephen Kraft, Raymond Buncic, Alex Levin

Research output: Contribution to journalArticle

7 Scopus citations

Abstract

Objective: To evaluate status of conjunctival fibrillin-1 in patients with Marfan syndrome with ectopia lentis. Methods: Frozen sections of conjunctiva from 6 patients with Marfan syndrome with ectopia lentis and from 15 age-matched control subjects were stained with mouse antihuman fibrillin-1 antibody, using an avidin biotin immunoperoxidase technique. The fibrillin-1 staining characteristics of conjunctiva were analyzed with the light microscope. Results: All the fresh frozen sections of conjunctival samples from control subjects demonstrated a characteristic pattern of fibrillin-1 staining. We observed a woven network of thin fibrils of uniform thickness surrounding collagen bundles. The fresh frozen samples from patients with Marfan syndrome showed consistent qualitative differences in fibrillin-1 staining when compared with samples from control subjects. The fibrils were longer and straighter than normal, varied in caliber, and showed fewer tendencies to form a woven pattern. Conclusions: Consistent, qualitative abnormalities in fibrillin-1 staining pattern can be seen in the conjunctiva of patients with Marfan syndrome with ectopia lentis. Conjunctival biopsy deserves further investigation as a diagnostic modality for Marfan syndrome in patients with ectopia lentis.

Original languageEnglish (US)
Pages (from-to)205-209
Number of pages5
JournalArchives of Ophthalmology
Volume124
Issue number2
DOIs
StatePublished - Feb 2006

ASJC Scopus subject areas

  • Ophthalmology

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    Ganesh, A., Smith, C., Chan, W., Unger, S., Quercia, N., Godfrey, M., Kraft, S., Buncic, R., & Levin, A. (2006). Immunohistochemical evaluation of conjunctival fibrillin-1 in Marfan syndrome. Archives of Ophthalmology, 124(2), 205-209. https://doi.org/10.1001/archopht.124.2.205