Ultrastructural properties of ciliary zonule microfibrils

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Ultrastructural properties of ciliary zonule microfibrils.

Conventional electron microscopy and rotary shadowing techniques have provided conflicting interpretations of microfibril ultrastructure. To address this issue, we have used quick-freeze deep-etch (QFDE) microscopy to obtain 3-dimensional surface views of microfibrils that have not been fixed, dehydrated, or stained with heavy metals. By this approach, microfibrils appear as tightly packed rows...

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Proteomic Analysis of the Bovine and Human Ciliary Zonule

Purpose The zonule of Zinn (ciliary zonule) is a system of fibers that centers the crystalline lens on the optical axis of the eye. Mutations in zonule components underlie syndromic conditions associated with a broad range of ocular pathologies, including microspherophakia and ectopia lentis. Here, we used HPLC-mass spectrometry to determine the molecular composition of the zonule. Methods Tr...

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Ultrastructural analyses of enzyme-treated microfibrils in rabbit corneal stroma.

Microfibrils have been identified within and between corneal collagen lamellae in a number of vertebrate species in a variety of developmental and pathological conditions, but they are relatively rare in normal adult animals. The present study was undertaken to analyze corneal microfibrils in adult rabbits using enzymatic digestion techniques. Transmission electron microscopy (TEM) showed clust...

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Development, composition, and structural arrangements of the ciliary zonule of the mouse.

PURPOSE Here, we examined the development, composition, and structural organization of the ciliary zonule of the mouse. Fibrillin 1, a large glycoprotein enriched in force-bearing tissues, is a prominent constituent of the mouse zonule. In humans, mutations in the gene for fibrillin 1 (FBN1) underlie Marfan syndrome (MS), a disorder characterized by lens dislocation and other ocular symptoms. ...

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Microfibril-Associated Glycoprotein-1 Controls Human Ciliary Zonule Development In Vitro

The ciliary zonule in the eye, also known as Zinn's zonule, is composed of oxytalan fibers, which are bundles of microfibrils consisting mainly of fibrillin-1. However, it is still unclear which of the microfibril-associated molecules present in the ciliary zonule controls oxytalan fibers. Microfibril-associated glycoprotein-1 (MAGP-1) is the only microfibril-associated molecule identified in t...

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ژورنال

عنوان ژورنال: Journal of Structural Biology

سال: 2002

ISSN: 1047-8477

DOI: 10.1016/s1047-8477(02)00559-2