Fibrillin and the eye
نویسندگان
چکیده
منابع مشابه
Fibrillin and the eye.
The glycoprotein fibrillin is the principal component of the ciliary zonule and has an important role in the strength and elasticity of ocular connective tissues. Fibrillin polymers form the structural scaVold of extensible microfibrils which are present in ocular elastic tissues and are arranged in parallel bundles to form the zonular fibres. These fibrillin-rich microfibrils are morphological...
متن کاملMatrix Metalloproteinase-2 Degrades Fibrillin-1 and Fibrillin-2 of Oxytalan Fibers in the Human Eye and Periodontal Ligaments In Vitro
Oxytalan fibers are distributed in the eye and periodontal ligaments (PDL). The ciliary zonule, known as Zinn's zonule, in the eye is composed of oxytalan fibers, which are bundles of microfibrils consisting mainly of fibrillin-1 and fibrillin-2. As turnover of oxytalan fibers is slow during life, their degradation mechanism remains unclarified. This study was performed to examine degradation p...
متن کاملHuman eye development is characterized by coordinated expression of fibrillin isoforms.
PURPOSE Mutations in human fibrillin-1 and -2, which are major constituents of tissue microfibrils, can affect multiple ocular components, including the ciliary zonule, lens, drainage apparatus, cornea, and retina. However, the expression pattern of the three human fibrillins and an integral microfibrillar component, MAGP1, during human eye development is not known. METHODS We analyzed sectio...
متن کاملFibrillin assembly requires fibronectin.
Fibrillins constitute the major backbone of multifunctional microfibrils in elastic and nonelastic extracellular matrices. Proper assembly mechanisms are central to the formation and function of these microfibrils, and their properties are often compromised in pathological circumstances such as in Marfan syndrome and in other fibrillinopathies. Here, we have used human dermal fibroblasts to ana...
متن کاملADAMTSL4, a secreted glycoprotein widely distributed in the eye, binds fibrillin-1 microfibrils and accelerates microfibril biogenesis.
PURPOSE ADAMTSL4 mutations cause autosomal recessive isolated ectopia lentis (IEL) and ectopia lentis et pupillae. Dominant FBN1 mutations cause IEL or syndromic ectopia lentis (Marfan syndrome and Weill-Marchesani syndrome). The authors sought to characterize recombinant ADAMTSL4 and the ocular distribution of ADAMTSL4 and to investigate whether ADAMTSL4 influences the biogenesis of fibrillin-...
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ژورنال
عنوان ژورنال: British Journal of Ophthalmology
سال: 2000
ISSN: 0007-1161
DOI: 10.1136/bjo.84.11.1312