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Fibulin-5/DANCE is essential for elastogenesis in vivo
Tomoyuki Nakamura1, Pilar Ruiz Lozano, Yasuhiro Ikeda
1UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego, La Jolla, California 92093, USA.
Nature
|January 24, 2002
Summary
Fibulin-5 is crucial for organizing elastic fibers, essential for the function of arteries, lungs, and skin. Mice lacking fibulin-5 show disorganized elastic fibers, leading to tissue damage and disease.
Area of Science:
- Biochemistry
- Developmental Biology
- Cell Biology
Background:
- Elastic fibers are vital for tissue elasticity in organs like arteries, lungs, and skin.
- The assembly and development of elastic fibers, composed of microfibrils and elastin, are not fully understood.
Purpose of the Study:
- To investigate the role of fibulin-5 (DANCE) in the organization of elastic fibers.
- To determine the function of fibulin-5 in the development and maintenance of elastic tissues.
Main Methods:
- Gene targeting in mice to create fibulin-5 knockout (fibulin-5-/-) models.
- In vitro studies to assess fibulin-5 interactions with elastic fibers and integrins.
- Histological and functional analysis of tissues from fibulin-5-/- mice.
Main Results:
- Fibulin-5 knockout mice exhibit severely disorganized elastic fibers throughout the body.
- These mice display aortic abnormalities, emphysema, and cutis laxa, indicating defective elastic fiber development.
- Fibulin-5 directly interacts with elastic fibers and binds to integrins alphavbeta3, alphavbeta5, and alpha9beta1.
Conclusions:
- Fibulin-5 is an essential determinant of elastic fiber organization and stability.
- It may function by anchoring elastic fibers to cells via integrin interactions.
- Defects in fibulin-5 lead to impaired elastic tissue structure and function, contributing to various pathologies.
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