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

• Published in: Micron (Oxford, England : 1993) Vol. 32; no. 2; pp. 185 - 200
• Main Authors: Sherratt, M.J., Wess, T.J., Baldock, C., Ashworth, J., Purslow, P.P., Shuttleworth, C.A., Kielty, C.M.
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01-02-2001
• Subjects: Atomic force microscopy; Ectopia Lentis - genetics; Elasticity; Environmental scanning electron microscopy; Extracellular Matrix Proteins - genetics; Extracellular Matrix Proteins - ultrastructure; Fibrillin; Fibrillins; Humans; Marfan Syndrome - genetics; Microfibrils; Microfibrils - ultrastructure; Microfilament Proteins - genetics; Microfilament Proteins - ultrastructure; Models, Structural; Scanning transmission electron microscopy; Supramolecular organisation; Transmission electron microscopy; Ultrastructure; Atomic force microscopy; Transmission electron microscopy; Ultrastructure; Environmental scanning electron microscopy; Microfibrils; Supramolecular organisation; Fibrillin; Scanning transmission electron microscopy
• ISSN: 0968-4328; 1878-4291
• DOI: 10.1016/S0968-4328(99)00082-7

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.