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Fibrillin-rich microfibrils of the extracellular matrix: Ultrastructure and assembly
Journal article   Peer reviewed

Fibrillin-rich microfibrils of the extracellular matrix: Ultrastructure and assembly

M J Sherratt, Tim J Wess, Clair Baldock, J Ashworth, P P Purslow, C A Shuttleworth and C M Kielty
Micron, Vol.32(2), pp.185-200
2001
url
https://doi.org/10.1016/S0968-4328(99)00082-7View
Published Version

Abstract

fibrillin microfibrils ultrastructure supramolecular organisation scanning transmission electron microscopy transmission electron microscopy environmental scanning electron microscopy atomic force microscopy
Fibrillin-rich microfibrils are a unique class of extensible connective tissue macromolecules. Their critical contribution to the establishment and maintenance of diverse extracellular matrices was underlined by the linkage of their principal structural component fibrillin to Marfan syndrome, a heritable connective tissue disorder with pleiotropic manifestations. Microscopy and preparative techniques have contributed substantially to the understanding of microfibril structure and function. The supramolecular organisation of microfibrillar assemblies in tissues has been examined by tissue sectioning and X-ray diffraction methods. Published findings are discussed and new information reported on the organisation of microfibrils in the ciliary zonular fibrils by environmental scanning electron microscopy. This review summarises microscopy and X-ray diffraction studies that are informing current understanding of the ultrastructure of fibrillin-rich microfibrils. (C) 2000 Published by Elsevier Science Ltd.

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