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斑马鱼作为肌肉萎缩症和先天性肌病的模型。

The zebrafish as a model for muscular dystrophy and congenital myopathy.

作者信息

Bassett David I, Currie Peter D

机构信息

Comparative and Developmental Genetics Section, Western General Hospital, Edingburgh, UK.

出版信息

Hum Mol Genet. 2003 Oct 15;12 Spec No 2:R265-70. doi: 10.1093/hmg/ddg279.

DOI:10.1093/hmg/ddg279
PMID:14504264
Abstract

The muscular dystrophies and congenital myopathies are inherited diseases of the skeletal muscle, which lead to a loss of muscle function and are often fatal. While many of the loci involved are already known, these conditions remain incurable, and genetic models are being developed in an effort to understand the pathological mechanisms involved. Recently several papers have shown that the zebrafish, which is now widely used in developmental genetic studies, will provide a useful addition to our toolkit in this regard. Here we describe these studies, including a zebrafish model of what is potentially the novel pathological mechanism of muscle attachment failure in Duchenne and other muscular dystrophies.

摘要

肌肉营养不良症和先天性肌病是骨骼肌的遗传性疾病,会导致肌肉功能丧失,且往往是致命的。虽然许多相关基因座已为人所知,但这些病症仍然无法治愈,目前正在开发遗传模型以努力了解其中涉及的病理机制。最近有几篇论文表明,斑马鱼目前广泛应用于发育遗传学研究,在这方面将为我们的工具集增添助力。在此我们描述这些研究,包括一种斑马鱼模型,该模型可能揭示杜兴氏肌营养不良症和其他肌肉营养不良症中肌肉附着失败这一全新病理机制。

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