| First Author | Zheng Q | Year | 2007 |
| Journal | Mol Cell Biol | Volume | 27 |
| Issue | 3 | Pages | 1083-95 |
| PubMed ID | 17130242 | Mgi Jnum | J:118290 |
| Mgi Id | MGI:3699062 | Doi | 10.1128/MCB.01330-06 |
| Citation | Zheng Q, et al. (2007) Molecular analysis of fibulin-5 function during de novo synthesis of elastic fibers. Mol Cell Biol 27(3):1083-95 |
| abstractText | 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. |
