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Human Molecular Genetics Advance Access originally published online on May 11, 2005
Human Molecular Genetics 2005 14(13):1727-1743; doi:10.1093/hmg/ddi179
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© The Author 2005. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org

Zebrafish as a model for caveolin-associated muscle disease; caveolin-3 is required for myofibril organization and muscle cell patterning

Susan J. Nixon1,2,3, Jeremy Wegner4, Charles Ferguson1,2,3, Pierre-François Méry1,2,3,{dagger}, John F. Hancock1, Peter D. Currie5, Brian Key3, Monte Westerfield4 and Robert G. Parton1,2,3,*

1Institute for Molecular Bioscience, 2Centre for Microscopy and Microanalysis and 3School of Biomedical Sciences, University of Queensland, Brisbane 4072, Australia, 4Institute of Neuroscience, University of Oregon, Eugene, OR 97403, USA and 5Victor Chang Cardiac Research Institute, Darlinghurst, NSW 2010, Australia

* To whom correspondence should be addressed. Tel: +61 733656468; Fax: +61 733654422; Email: r.parton{at}imb.uq.edu.au

Received March 22, 2005; Accepted May 2, 2005

Caveolae are an abundant feature of many animal cells. However, the exact function of caveolae remains unclear. We have used the zebrafish, Danio rerio, as a system to understand caveolae function focusing on the muscle-specific caveolar protein, caveolin-3 (Cav3). We have identified caveolin-1 ({alpha} and ß), caveolin-2 and Cav3 in the zebrafish. Zebrafish Cav3 has 72% identity to human CAV3, and the amino acids altered in human muscle diseases are conserved in the zebrafish protein. During embryonic development, cav3 expression is apparent by early segmentation stages in the first differentiating muscle precursors, the adaxial cells and slightly later in the notochord. cav3 expression appears in the somites during mid-segmentation stages and then later in the pectoral fins and facial muscles. Cav3 and caveolae are located along the entire sarcolemma of late stage embryonic muscle fibers, whereas ß-dystroglycan is restricted to the muscle fiber ends. Down-regulation of Cav3 expression causes gross muscle abnormalities and uncoordinated movement. Ultrastructural analysis of isolated muscle fibers reveals defects in myoblast fusion and disorganized myofibril and membrane systems. Expression of the zebrafish equivalent to a human muscular dystrophy mutant, CAV3P104L, causes severe disruption of muscle differentiation. In addition, knockdown of Cav3 resulted in a dramatic up-regulation of eng1a expression resulting in an increase in the number of muscle pioneer-like cells adjacent to the notochord. These studies provide new insights into the role of Cav3 in muscle development and demonstrate its requirement for correct intracellular organization and myoblast fusion.

{dagger} Present address: Inserm U469, Endocrinologie Moléculaire: Signalisation Cellulaire et Pathologie, CCIPE, 141 rue de la Cardonille, 34094 Montpellier Cedex 5, France.


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