Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology
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
Rights Notice
Refer to copyright notice on published article.


Creation Date: 2010-12-01 12:00:00
Last Modified: 0001-01-01 12:00:00
An error has occurred. This application may no longer respond until reloaded. Reload 🗙