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  Vol. 120 No. 6, June 2002 TABLE OF CONTENTS
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  •  Online Features
  Clinicopathologic Reports, Case Reports, and Small Case Series
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Lens Dislocation in Marfan Syndrome: Potential Role of Matrix Metalloproteinases in Fibrillin Degradation

Arch Ophthalmol. 2002;120:833-835.

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Marfan syndrome is an autosomal dominant disorder with pleiotropic manifestations that involve the ocular, cardiovascular, and skeletal systems. Marfan syndrome remains primarily a clinical diagnosis with a frequency of 2 to 3 individuals per 10 000. Patients with this disorder may have a variety of ocular complaints, most commonly, subluxation of the lens, which occurs in more than 60% of patients.1 Several studies have identified the FBN1 fibrillin gene located on chromosome 15 as defective in this syndrome.2

Matrix metalloproteinases (MMPs) are proteolytic enzymes important in physiological and pathological remodeling, the activity of which is stringently controlled by a family of natural antagonists, the tissue inhibitors of MMPs (TIMPs). Both MMPs and TIMPs are present in the aqueous humor in normal and inflamed eyes,3 resulting in their interaction with the lens zonules.

We describe a patient with Marfan syndrome lens subluxation associated with positive MMP expression and no TIMP immunoreactivity within . . . [Full Text of this Article]

Report of a Case


Comment
Corresponding author: Minas T. Coroneo, FRACO, FRACS, MD, Department of Ophthalmology, Prince of Wales Hospital, University of New South Wales, Randwick, Sydney, Australia (e-mail: m.coroneo@unsw.edu.au).



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The molecular genetics of Marfan syndrome and related disorders
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Matrix Metalloproteinases and Tissue Inhibitors of Matrix Metalloproteinases in the Human Lens: Implications for Cortical Cataract Formation
Sachdev et al.
IOVS 2004;45:4075-4082.
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Isolated Ectopia Lentis: Potential Role of Matrix Metalloproteinases in Fibrillin Degradation
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Arch Ophthalmol 2004;122:111-114.
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Lens Dislocation in Marfan Syndrome and UV-B Light Exposure
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Arch Ophthalmol 2003;121:585-585.
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