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Fibrillin microfibrils in bone physiology

Silvia Smaldone1, Francesco Ramirez1

  • 1Department of Pharmacology and Systems Therapeutics, Institute for Systems Biomedicine, Icahn School of Medicine at Mount Sinai, New York, NY 10029, United States.

Matrix Biology : Journal of the International Society for Matrix Biology
|September 27, 2015
PubMed

Insights

Fibrillin proteins are crucial for bone development and function, impacting skeletal abnormalities in Marfan syndrome and congenital contractural arachnodactyly. Their roles in signaling pathways are key to understanding bone degeneration.

Area of Science:

  • Skeletal Biology
  • Connective Tissue Biology
  • Developmental Biology

Background:

  • Fibrillin assemblies (microfibrils and elastic fibers) are vital for bone formation and function, despite low abundance in skeletal matrices.
  • Severe skeletal abnormalities in Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCA) highlight the importance of fibrillins.

Purpose of the Study:

  • To investigate the distinct roles of fibrillin-1 and fibrillin-2 in bone development and metabolism.
  • To elucidate the pathophysiological mechanisms linking fibrillin mutations to skeletal phenotypes.
  • To understand the contribution of fibrillin scaffolds to TGFβ and BMP signaling in bone.

Main Methods:

  • Utilizing mouse models of MFS and CCA to study skeletal phenotypes.
  • Analyzing the distinct contributions of fibrillin-1 and fibrillin-2 to signaling pathways.
  • Examining the impact on marrow mesenchymal stem cell commitment and differentiation.

Main Results:

  • Fibrillin-2 uniquely regulates BMP-dependent limb patterning.
  • Fibrillin-1 and fibrillin-2 have distinct effects on mesenchymal stem cell fate.
  • Mutant mice exhibit skeletal phenotypes linked to specific pathophysiological mechanisms.

Conclusions:

  • Fibrillin scaffolds critically influence bone patterning, growth, and metabolism via TGFβ and BMP signaling.
  • Understanding fibrillin function is essential for addressing age- and injury-related bone degeneration.
Keywords:
Autopod patterningBone marrow nicheFibrillinMarfan syndromeMesenchymal stem cellsOsteopeniaTGFβ and BMP signaling

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