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Fibrillin-rich microfibrils of the extracellular matrix: ultrastructure and assembly
M J Sherratt1, T J Wess, C Baldock
1School of Biological Sciences, 2.205 Stopford Building, University of Manchester M13 9PT, Manchester, UK. msherrat@fs1.scg.man.ac.uk
Abstract:
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.
Insights
Fibrillin-rich microfibrils are key to connective tissues and their disorders. This review details microscopy and X-ray diffraction findings on microfibril ultrastructure and organization.
Area of Science:
- Connective Tissue Biology
- Biomolecular Structure and Function
- Extracellular Matrix Research
Background:
- Fibrillin-rich microfibrils are essential extensible macromolecules in connective tissues.
- Defects in fibrillin, the main component, cause Marfan syndrome, a complex genetic disorder.
- Understanding microfibril structure is crucial for comprehending tissue integrity and disease pathogenesis.
Purpose of the Study:
- To summarize current knowledge on the ultrastructure of fibrillin-rich microfibrils.
- To review findings from microscopy and X-ray diffraction studies.
- To present new data on ciliary zonular fibril organization using environmental scanning electron microscopy.
Main Methods:
- Analysis of fibrillin-rich microfibril structure and organization.
- Application of microscopy techniques, including environmental scanning electron microscopy.
- Utilisation of X-ray diffraction and tissue sectioning methods for supramolecular analysis.
Main Results:
- Microscopy and preparative techniques have significantly advanced the understanding of microfibril structure and function.
- Tissue sectioning and X-ray diffraction have elucidated the supramolecular organization of microfibrillar assemblies.
- New insights into the organization of microfibrils within ciliary zonular fibrils have been obtained.
Conclusions:
- Fibrillin-rich microfibrils play a vital role in diverse extracellular matrices.
- Integrated microscopy and diffraction studies provide a comprehensive view of microfibril ultrastructure.
- Continued research is essential for fully understanding microfibril contributions to tissue health and disease.