Unraveling the mechanism of elastic fiber assembly: The roles of short fibulins

Hiromi Yanagisawa, Elaine C. Davis

Research output: Contribution to journalReview article

72 Scopus citations

Abstract

Evolution of elastic fibers is associated with establishment of the closed circulation system. Primary roles of elastic fibers are to provide elasticity and recoiling to tissues and organs and to maintain the structural integrity against mechanical strain over a lifetime. Elastic fibers are comprised of an insoluble elastin core and surrounding mantle of microfibrils. Elastic fibers are formed in a regulated, stepwise manner, which includes the formation of a microfibrillar scaffold, deposition and integration of tropoelastin monomers into the scaffold, and cross-linking of the monomers to form an insoluble, functional polymer. In recent years, an increasing number of glycoproteins have been identified and shown to be located on or surrounding elastic fibers. Among them, the short fibulins-3, -4 and -5 particularly drew attention because of their potent elastogenic activity. Fibulins-3, -4 and -5 are characterized by tandem repeats of calcium binding EGF-like motifs and a C-terminal fibulin module, which is conserved throughout fibulin family members. Initial biochemical characterization and gene expression studies predicted that fibulins might be involved in structural support and/or matrix-cell interactions. Recent analyses of short fibulin knockout mice have revealed their critical roles in elastic fiber development in vivo. We review recent findings on the elastogenic functions of short fibulins and discuss the molecular mechanism underlying their activity in vitro and in vivo.

Original languageEnglish (US)
Pages (from-to)1084-1093
Number of pages10
JournalInternational Journal of Biochemistry and Cell Biology
Volume42
Issue number7
DOIs
StatePublished - Jul 2010

Keywords

  • Assembly
  • Elastic fibers
  • Knockout mouse
  • Lysyl oxidase
  • Microfibrils

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology

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