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First published online October 22, 2003

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Fish model for Duchenne muscular dystrophy

In zebrafish, the recessive lethal mutation sapje causes progressive degeneration of skeletal muscles, reminiscent of human muscular dystrophies. Bassett and co-workers show that the sapje mutation disrupts the zebrafish orthologue of the human Duchenne muscular dystrophy (DMD) gene (see p. 5851). In a detailed study of the structure of the muscles throughout embryonic development, the researchers report that the progressive muscle degeneration in the mutant fish is caused by the separation of somitic fibres from their attachment points on tendon-like sheets of extracellular matrix, called myosepta. These attachment points resemble mammalian myomuscular and myotendinous junctions. However, although structural deficits of myotendinous junctions have been seen in mouse models of Duchenne muscular dystrophy, dystrophin has not previously been implicated in the maintenance of either structure. Thus, say the researchers, the sapje mutant may be a good model for a hitherto unsuspected pathological mechanism in muscular dystrophies.

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Dystrophin is required for the formation of stable muscle attachments in the zebrafish embryo

David I. Bassett, Robert J. Bryson-Richardson, David F. Daggett, Philippe Gautier, David G. Keenan, and Peter D. Currie
Development 2003 130: 5851-5860. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in Development Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content