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First published online September 7, 2009
doi: 10.1242/10.1242/dev.034561

Development 136, 3367-3376 (2009)
Published by The Company of Biologists 2009
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The zebrafish dystrophic mutant softy maintains muscle fibre viability despite basement membrane rupture and muscle detachment

Arie S. Jacoby1, Elisabeth Busch-Nentwich2, Robert J. Bryson-Richardson1,3,6, Thomas E. Hall1,6, Joachim Berger1,6, Silke Berger1,6, Carmen Sonntag1,6, Caroline Sachs1, Robert Geisler4, Derek L. Stemple2 and Peter D. Currie1,5,6,*

1 The Victor Chang Cardiac Research Institute, Darlinghurst, NSW 2010, Australia.
2 The Wellcome Trust Sanger Institute, Cambridge CB10 1SA, UK.
3 School of Medical Sciences, Faculty of Medicine, University of New South Wales, NSW 2065, Australia.
4 Max-Planck Institute for Developmental Biology, Tübingen 72076, Germany.
5 St Vincent's Clinical School, Faculty of Medicine, University of New South Wales, NSW 2010, Australia.
6 Australian Regenerative Medicine Institute, Monash University, VIC 3800, Australia.

* Author for correspondence (peter.currie{at}armi.monash.edu.au)

Accepted 28 July 2009

The skeletal muscle basement membrane fulfils several crucial functions during development and in the mature myotome and defects in its composition underlie certain forms of muscular dystrophy. A major component of this extracellular structure is the laminin polymer, which assembles into a resilient meshwork that protects the sarcolemma during contraction. Here we describe a zebrafish mutant, softy, which displays severe embryonic muscle degeneration as a result of initial basement membrane failure. The softy phenotype is caused by a mutation in the lamb2 gene, identifying laminin β2 as an essential component of this basement membrane. Uniquely, softy homozygotes are able to recover and survive to adulthood despite the loss of myofibre adhesion. We identify the formation of ectopic, stable basement membrane attachments as a novel means by which detached fibres are able to maintain viability. This demonstration of a muscular dystrophy model possessing innate fibre viability following muscle detachment suggests basement membrane augmentation as a therapeutic strategy to inhibit myofibre loss.

Key words: Skeletal muscle, Zebrafish, Laminin β2, Basement membrane, Muscular dystrophy


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© The Company of Biologists Ltd 2009