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Extracellular matrix 4: the elastic fiber

J Rosenbloom1, W R Abrams, R Mecham

  • 1Department of Anatomy and Histology, School of Dental Medicine, University of Pennsylvania, Philadelphia 19104.

FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology
|October 1, 1993
PubMed
Summary

Elastic fibers, crucial for tissue elasticity, consist of amorphous elastin and microfibrils. Mutations in fibrillin genes cause Marfan syndrome, highlighting the importance of these extracellular matrix components.

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Area of Science:

  • Biochemistry
  • Molecular Biology
  • Tissue Engineering

Background:

  • Elastic fibers provide essential mechanical properties to tissues like lungs and blood vessels.
  • These fibers comprise an amorphous elastin core and a peripheral microfibrillar component.

Purpose of the Study:

  • To elucidate the structure and function of elastic fibers.
  • To investigate the molecular basis of elastin and microfibril formation.
  • To explore the genetic underpinnings of related connective tissue disorders.

Main Methods:

  • Biochemical analysis to determine protein composition.
  • Ultrastructural analysis to visualize fiber organization.
  • Genetic analysis of elastin and fibrillin genes.

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Main Results:

  • Elastin, a hydrophobic protein rich in glycine and proline, forms the amorphous core.
  • Tropoelastin, the 72-kDa precursor, is cross-linked by lysyl oxidase into an elastic network.
  • Microfibrils, containing fibrillin, act as a scaffold for elastin deposition.
  • Alternative splicing generates multiple tropoelastin isoforms.
  • Mutations in the fibrillin gene (chromosome 15) are linked to Marfan syndrome.

Conclusions:

  • Elastin and microfibrils are key components of the extracellular matrix, essential for tissue elasticity.
  • Complex transcriptional regulation governs elastin gene expression.
  • Fibrillin's role in organizing elastic fibers is critical.
  • Genetic defects in fibrillin are causative for Marfan syndrome.