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Fibulin-5/DANCE is essential for elastogenesis in vivo

Fibulin-5/DANCE is essential for elastogenesis in vivo,10.1038/415171a,Nature,Tomoyuki Nakamura,Pilar Ruiz Lozano,Yasuhiro Ikeda,Yoshitaka Iwanaga,Ale

Fibulin-5/DANCE is essential for elastogenesis in vivo   (Citations: 175)
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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 Jr.http://academic.research.microsoft.com/io.ashx?type=5&id=14890371&selfId1=0&selfId2=0&maxNumber=12&query=
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 skin. Although elastic fibres are known to be composed of microfibril proteins (for example, fibrillins and latent transforming growth factor (TGF)-beta-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 ligand, 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 alphavbeta3, alphavbeta5 and alpha9beta1 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.
Journal: Nature , vol. 415, no. 6868, pp. 171-175, 2002
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    • ...For example, a fibulin-5 KO mouse model has been used to show that defective organization and stabilization of elastic fibers produces stiffer arteries [...

    Craig J. Goergenet al. Induced Chromosome Deletion in a Williams-Beuren Syndrome Mouse Model ...

    • ...Via its RGD motif fibulin-5 binds various integrins, such as a4b1, a5b1, a9b1, avb3 and avb5 and thereby mediates cell adhesion (Nakamura et al. 1999, 2002; Lomas et al. 2007; Timpl et al. 2003)...
    • ...Moreover, fibulin-5 interacts with the elastic fibre molecules tropoelastin and fibrillin-1 (Freeman et al. 2005; Zheng et al. 2007; Nakamura et al. 2002) and thus was implicated to be important for elastic fibre formation (Yanagisawa et al. 2002; Nakamura et al. 2002)...
    • ...Moreover, fibulin-5 interacts with the elastic fibre molecules tropoelastin and fibrillin-1 (Freeman et al. 2005; Zheng et al. 2007; Nakamura et al. 2002) and thus was implicated to be important for elastic fibre formation (Yanagisawa et al. 2002; Nakamura et al. 2002)...
    • ...Fbln5 knockout mice survive to adulthood but show systemic elastic fibre defects, causing loose skin, emphysema, tortuous blood vessels and increased arterial stiffness (Nakamura et al. 2002; Yanagisawa et al. 2002)...

    Martin GausterVeronikaet al. Fibulin-5 expression in the human placenta

    • ... It has been previously demonstrated that fibulin-4 and -5 are in charge of recruiting tropoelastins and its cross-linking enzymes onto microfibrils in order to accelerate elastic fiber assembly in collaboration with LTBP-2, which binds to heparin and heparin sulfate proteoglycan...

    Shinya Kasamatsuet al. Essential role of microfibrillar-associated protein 4 in human cutaneo...

    • ...In vitro analyses have implicated MAGP-1 and fibulin-5 in this process as bridging molecules between tropoelastin and fibrillins and between them and integrin receptors, respectively (Jensen et al. 2001; Yanagisawa et al. 2002; Nakamura et al. 2002; Rock et al. 2004; Freeman et al. 2005; Lomas et al. 2007; El-Hallous et al. 2007)...

    Francesco Ramirezet al. Biogenesis and function of fibrillin assemblies

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