McArdle A, Edwards R H, Jackson M J
Muscle Research Centre, Department of Medicine, University of Liverpool, UK.
Neuromuscul Disord. 1995 Nov;5(6):445-56. doi: 10.1016/0960-8966(95)00001-4.
The mdx mouse has a defect in the same gene as boys with Duchenne muscular dystrophy, which results in the absence of the protein product, dystrophin. A large number of recent studies have used the mdx mouse model to examine the potential role of dystrophy in normal muscle and the mechanisms by which dystrophin-deficiency leads to myopathy. This review discusses critically the results of these studies and their relevance to understanding the mechanisms by which dystrophin-deficiency leads to muscle necrosis.
mdx小鼠与患有杜兴氏肌营养不良症的男孩在同一个基因上存在缺陷，这导致蛋白质产物抗肌萎缩蛋白缺失。最近大量研究使用mdx小鼠模型来研究肌营养不良在正常肌肉中的潜在作用，以及抗肌萎缩蛋白缺乏导致肌病的机制。本综述批判性地讨论了这些研究的结果及其与理解抗肌萎缩蛋白缺乏导致肌肉坏死机制的相关性。
