Molecular analysis of fibulin-5 function during de novo synthesis of elastic fibers

Mol Cell Biol. 2007 Feb;27(3):1083-95. doi: 10.1128/MCB.01330-06. Epub 2006 Nov 27.

Abstract

Elastic fibers contribute to the structural support of tissues and to the regulation of cellular behavior. Mice deficient for the fibulin-5 gene (fbln5(-/-)) were used to further elucidate the molecular mechanism of elastic fiber assembly. Major elastic fiber components were present in the skin of fbln5(-/-) mice despite a dramatic reduction of mature elastic fibers. We found that fibulin-5 preferentially bound the monomeric form of elastin through N-terminal and C-terminal elastin-binding regions and to a preexisting matrix scaffold through calcium-binding epidermal growth factor (EGF)-like (CB-EGF) domains. We further showed that adenovirus-mediated gene transfer of fbln5 was sufficient to regenerate elastic fibers and increase elastic fiber-cell connections in vivo. A mutant fibulin-5 lacking the first 28 amino acids of the first CB-EGF domain, however, was unable to rescue elastic fiber defects. Fibulin-5 thus serves as an adaptor molecule between monomeric elastin and the matrix scaffold to aid in elastic fiber assembly. These results also support the potential use of fibulin-5 as a therapeutic agent for the treatment of elastinopathies.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenoviridae
  • Animals
  • Animals, Newborn
  • CHO Cells
  • COS Cells
  • Chlorocebus aethiops
  • Chondrocytes / cytology
  • Cricetinae
  • Cricetulus
  • Dermis / cytology
  • Elastic Tissue / cytology*
  • Elastic Tissue / metabolism*
  • Elasticity
  • Elastin / genetics
  • Epidermal Growth Factor / metabolism
  • Extracellular Matrix / metabolism
  • Extracellular Matrix Proteins / chemistry
  • Extracellular Matrix Proteins / metabolism*
  • Fibroblasts / cytology
  • Gene Transfer Techniques
  • Protein Binding
  • Protein Structure, Tertiary
  • Recombinant Proteins / chemistry
  • Recombinant Proteins / metabolism*
  • Solubility

Substances

  • Extracellular Matrix Proteins
  • Fbln5 protein, mouse
  • Fbln5 protein, rat
  • Recombinant Proteins
  • Epidermal Growth Factor
  • Elastin