Material and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils

Emilio Arteaga-Solis, Lee Sui-Arteaga, Minwook Kim, Mitchell B. Schaffler, Karl J. Jepsen, Nancy Pleshko, Francesco Ramirez

Research output: Contribution to journalArticlepeer-review

21 Scopus citations

Abstract

The contribution of non-collagenous components of the extracellular matrix to bone strength is largely undefined. Here we report that deficiency of fibrillin-1 or fibrillin-2 microfibrils causes distinct changes in bone material and mechanical properties. Morphometric examination of mice with hypomorphic or null mutations in fibrillin-1 or fibrillin-2, respectively, revealed appreciable differences in the postnatal shaping and growth of long bones. Fourier transform infrared imaging spectroscopy indicated that fibrillin-1 plays a predominantly greater role than fibrillin-2 in determining the material properties of bones. Biomechanical tests demonstrated that fibrillin-2 exerts a greater positive influence on the mechanical properties of bone than fibrillin-1 assemblies. Published evidence indirectly supports the notion that the above findings are mostly, if not exclusively, related to the differential control of TGFβ family signaling by fibrillin proteins. Our study therefore advances our understanding of the role that extracellular microfibrils play in bone physiology and implicitly, in the pathogenesis of bone loss in human diseases caused by mutations in fibrillin-1 or -2.

Original languageEnglish
Pages (from-to)188-194
Number of pages7
JournalMatrix Biology
Volume30
Issue number3
DOIs
StatePublished - Apr 2011

Keywords

  • Bone material and mechanical properties
  • Congenital contractural arachnodactyly
  • Fibrillin
  • Marfan syndrome
  • TGFβ

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