Human Molecular Genetics Advance Access published online on May 11, 2005
Human Molecular Genetics, doi:10.1093/hmg/ddi179
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1 Institute for Molecular Bioscience, University of Queensland, Brisbane 4072, Australia; Centre for Microscopy and Microanalysis, University of Queensland, Brisbane 4072, Australia; School of Biomedical Sciences, University of Queensland, Brisbane 4072, Australia
* To whom correspondence should be addressed. 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 (
Received March 22, 2005
Revised April 28, 2005
Accepted April 28, 2005
Article
Zebrafish as a model for caveolin-associated muscle disease; caveolin-3 is required for myofibril organization and muscle cell patterning
2 Institute of Neuroscience, University of Oregon, Eugene, Oregon, 97403 USA
3 Institute for Molecular Bioscience, University of Queensland, Brisbane 4072, Australia
4 Victor Chang Cardiac Research Institute, Darlinghurst, NSW, 2010 Australia
5 School of Biomedical Sciences, University of Queensland, Brisbane 4072, Australia
Robert G. Parton, E-mail: r.parton{at}imb.uq.edu.au
Abstract 
and 
), caveolin-2 and caveolin-3 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 -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 of a human muscular dystrophy mutant, CAV3P104L, causes severe disruption of muscle differentiation. In addition, knock-down of caveolin-3 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.
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