Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology

Berger, Joachim, Berger, Silke, Hall, Thomas E., Lieschke, Graham J. and Currie, Peter D. (2010) Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology. Neuromuscular Disorders, 20 12: 826-832. doi:10.1016/j.nmd.2010.08.004

Author Berger, Joachim
Berger, Silke
Hall, Thomas E.
Lieschke, Graham J.
Currie, Peter D.
Title Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology
Journal name Neuromuscular Disorders   Check publisher's open access policy
ISSN 0960-8966
Publication date 2010-12-01
Sub-type Article (original research)
DOI 10.1016/j.nmd.2010.08.004
Volume 20
Issue 12
Start page 826
End page 832
Total pages 7
Place of publication Oxford, United Kingdom
Publisher Elsevier
Language eng
Formatted abstract
Duchenne muscular dystrophy is caused by mutations in the dystrophin gene. As in humans, zebrafish dystrophin is initially expressed at the peripheral ends of the myofibres adjacent to the myotendinous junction and gradually shifts to non-junctional sites. Dystrophin-deficient zebrafish larvae are characterised by abundant necrotic fibres being replaced by mono-nucleated infiltrates, extensive fibrosis accompanied by inflammation, and a broader variation in muscle fibre cross-sectional areas. Muscle progenitor proliferation cannot compensate for the extensive skeletal muscle loss. Live imaging of dystrophin-deficient zebrafish larvae documents detaching myofibres elicited by muscle contraction. Correspondingly, the progressive phenotype of dystrophin-deficient zebrafish resembles many aspects of the human disease, suggesting that specific advantages of the zebrafish model system, such as the ability to undertake in vivo drug screens and real time analysis of muscle fibre loss, could be used to make novel insights relevant to understanding and treating the pathological basis of dystrophin-deficient muscular dystrophy.
Keyword Dystrophin
Duchenne muscular dystrophy
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: ERA 2012 Admin Only
Institute for Molecular Bioscience - Publications
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Created: Tue, 07 Feb 2012, 20:20:28 EST by Susan Allen on behalf of Institute for Molecular Bioscience