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

被引：70

作者：

Berger, Joachim ^{[1
]}

Berger, Silke ^{[1
]}

Hall, Thomas E. ^{[1
]}

Lieschke, Graham J. ^{[2
]}

Currie, Peter D. ^{[1
]}

机构：

[1] Monash Univ, Australian Regenerat Med Inst, Clayton, Vic 3800, Australia

[2] Walter & Eliza Hall Inst Med Res, Canc & Haematol Div, Parkville, Vic 3052, Australia

来源：

NEUROMUSCULAR DISORDERS | 2010年 / 20卷 / 12期

基金：

英国医学研究理事会;

关键词：

Dystrophin; DMD; Duchenne muscular dystrophy; Zebrafish; Muscle; HUMAN SKELETAL-MUSCLE; ADULT ZEBRAFISH; EMBRYO; PATHOGENESIS; CALCIUM; CELLS; GENE; MICE;

D O I：

10.1016/j.nmd.2010.08.004

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

100204 [神经病学];

摘要：

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

引用

页码：826 / 832

页数：7

共 27 条

[1]

Calcium and the damage pathways in muscular dystrophy [J].