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

Fibrillin-rich microfibrils are a unique class of extensible connective tis sue macromolecules. Their critical contribution to the establishment and ma intenance of diverse extracellular matrices was underlined by the linkage o f their principal structural component fibrillin to Marfan syndrome, a heri table connective tissue disorder with pleiotropic manifestations. Microscop y and preparative techniques have contributed substantially to the understa nding of microfibril structure and function. The supramolecular organisatio n of microfibrillar assemblies in tissues has been examined by tissue secti oning and X-ray diffraction methods. Published findings are discussed and n ew 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 curr ent understanding of the ultrastructure of fibrillin-rich microfibrils. (C) 2000 Published by Elsevier Science Ltd.