Fibrillin: from microfibril assembly to biomechanical function

Kielty, C.M.; Baldock, C.; Lee, D.; Rock, M.J.; Ashworth, J.L.; Shuttleworth, C.A.

Philosophical Transactions of the Royal Society of London. Series B Biological Sciences 357(1418): 207-217

2002


ISSN/ISBN: 0962-8436
PMID: 11911778
DOI: 10.1098/rstb.2001.1029
Accession: 010663033

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Abstract
Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long-range elasticity. Their biological importance is emphasized by the linkage of fibrillin mutations to Marfan syndrome and related connective tissue disorders, which are associated with severe cardiovascular, ocular and skeletal defects. These microfibrils have a complex ultrastructure and it has proved a major challenge both to define their structural organization and to relate it to their biological function. However, new approaches have at last begun to reveal important insights into their molecular assembly, structural organization and biomechanical properties. This paper describes the current understanding of the molecular assembly of fibrillin molecules, the alignment of fibrillin molecules within microfibrils and the unique elastomeric properties of microfibrils.