Fibulin-5/DANCE is essential for elastogenesis in vivo

Tomoyuki Nakamura, Pilar Ruiz Lozano, Yasuhiro Ikeda, Yoshitaka Iwanaga, Aleksander Hinek, Susumu Minamisawa, Ching-Feng Cheng, Kazuhiro Kobuke, Nancy Dalton, Yoshikazu Takada, Kei Tashiro, John Ross, Tasuku Honjo and Kenneth R. Chien ()
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Tomoyuki Nakamura: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Pilar Ruiz Lozano: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Yasuhiro Ikeda: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Yoshitaka Iwanaga: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Aleksander Hinek: The Hospital for Sick Children
Susumu Minamisawa: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Ching-Feng Cheng: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Kazuhiro Kobuke: Graduate School of Medicine, Kyoto University
Nancy Dalton: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Yoshikazu Takada: Vascular Biology, the Scripps Research Institute
Kei Tashiro: Center for Molecular Biology and Genetics, Kyoto University
John Ross: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego
Tasuku Honjo: Graduate School of Medicine, Kyoto University
Kenneth R. Chien: UCSD-Salk Program in Molecular Medicine and the UCSD Institute of Molecular Medicine, University of California, San Diego

Nature, 2002, vol. 415, issue 6868, 171-175

Abstract: Abstract The elastic fibre system has a principal role in the structure and function of various types of organs that require elasticity, such as large arteries, lung and skin1,2. Although elastic fibres are known to be composed of microfibril proteins (for example, fibrillins and latent transforming growth factor (TGF)-β-binding proteins) and polymerized elastin, the mechanism of their assembly and development is not well understood. Here we report that fibulin-5 (also known as DANCE), a recently discovered integrin ligand3, is an essential determinant of elastic fibre organization. fibulin-5-/- mice generated by gene targeting exhibit a severely disorganized elastic fibre system throughout the body. fibulin-5-/- mice survive to adulthood, but have a tortuous aorta with loss of compliance, severe emphysema, and loose skin (cutis laxa). These tissues contain fragmented elastin without an increase of elastase activity, indicating defective development of elastic fibres. Fibulin-5 interacts directly with elastic fibres in vitro, and serves as a ligand for cell surface integrins αvβ3, αvβ5 and α9β1 through its amino-terminal domain. Thus, fibulin-5 may provide anchorage of elastic fibres to cells, thereby acting to stabilize and organize elastic fibres in the skin, lung and vasculature.

Date: 2002
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DOI: 10.1038/415171a

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