Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology
- Author(s)
- Berger, J; Berger, S; Hall, TE; Lieschke, GJ; Currie, PD;
- Details
- Publication Year 2010-12,Volume 20,Issue #12,Page 826-832
- Journal Title
- NEUROMUSCULAR DISORDERS
- Publication Type
- Journal Article
- 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 (C) 2010 Elsevier B V All rights reserved
- Publisher
- PERGAMON-ELSEVIER SCIENCE LTD
- Keywords
- HUMAN SKELETAL-MUSCLE; ADULT ZEBRAFISH; EMBRYO; PATHOGENESIS; CALCIUM; CELLS; GENE; MICE
- Publisher's Version
- https://doi.org/10.1016/j.nmd.2010.08.004
- Terms of Use/Rights Notice
- Refer to copyright notice on published article.
Creation Date: 2010-12-01 12:00:00