Fibrillin microfibrils: Multipurpose extracellular networks in organismal physiology

Francesco Ramirez, Lynn Y. Sakai, Harry C. Dietz, Daniel B. Rifkin

Research output: Contribution to journalReview article

93 Scopus citations

Abstract

Organismal physiology depends significantly on the proper assembly of extracellular matrix (ECM) macroaggregates that impart structural integrity to the connective tissue. Recent genetic studies in mice have unraveled unsuspected new functions of architectural matrix components in regulating signaling events that modulate patterning, morphogenesis, and growth of several organ systems. As a result, a new paradigm has emerged whereby tissue-specific organization of the ECM dictates not only the physical properties of the connective tissue, but also the ability of the matrix to direct a broad spectrum of cellular activities through the regulation of growth factor signaling. These observations pave the way to novel therapeutic approaches aimed at counteracting the deleterious consequences of perturbations of connective tissue homeostasis.

Original languageEnglish (US)
Pages (from-to)151-154
Number of pages4
JournalPhysiological genomics
Volume19
DOIs
StatePublished - Jan 1 2005

Keywords

  • Elastic fiber
  • Growth factor signaling
  • Marfan syndrome

ASJC Scopus subject areas

  • Physiology
  • Genetics

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