Fibrillin: From microfibril assembly to biomechanical function

Cay M. Kielty, Clair Baldock, David Lee, Matthew J. Rock, Jane L. Ashworth, C. Adrian Shuttleworth

Research output: Contribution to journalArticlepeer-review

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 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 elastometric properties of microfibrils.
Original languageEnglish
Pages (from-to)207-217
Number of pages10
JournalPhilosophical Transactions of the Royal Society B: Biological Sciences
Volume357
Issue number1418
DOIs
Publication statusPublished - 28 Feb 2002

Keywords

  • Extensibility
  • Fibrillin assembly
  • Microfibrils
  • Organization

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