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Lens Dislocation in Marfan Syndrome: Potential Role of Matrix Metalloproteinases in Fibrillin Degradation
Arch Ophthalmol. 2002;120:833-835.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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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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