ULTRASTRUCTURE OF THE SKELETAL-MUSCLE IN THE X-CHROMOSOME-LINKED DYSTROPHIC (MDX) MOUSE - COMPARISON WITH DUCHENNE MUSCULAR-DYSTROPHY
ULTRASTRUCTURE OF THE SKELETAL-MUSCLE IN THE X-CHROMOSOME-LINKED DYSTROPHIC (MDX) MOUSE - COMPARISON WITH DUCHENNE MUSCULAR-DYSTROPHY
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DOI：
10.1007/bf00688245
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发表时间：
1988-01-01
影响因子：
12.7
通讯作者：
JAROS, E
中科院分区：
文献类型：
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作者：
CULLEN, MJ;JAROS, E
X染色体连锁的小鼠肌营养不良症（mdx）和Duchenne型肌营养不良症（DMD）在超微结构上有明显的区别。特别是小鼠肌肉不会被结缔组织的聚集体浸润。有人提出，这种差异是由于在两种情况下肌营养不良蛋白的缺乏引起的继发性生化变化。如果是这种情况，应注意这两种疾病共同的最早期超微结构变化。其中最明显的是肌浆网的扩张，先于肌原纤维的断裂。这与缺乏抗肌萎缩蛋白之间的任何生理联系仍有待确定。我们认为，在mdx小鼠，广泛的肌纤维坏死发生在3-4周是由机械需求增加引起的肌营养不良蛋白的缺乏，成为关键在这个时候。随后的肌纤维再生似乎几乎完全成功。相比之下，DMD中再生的最终失败可能是由于在DMD中起作用的其他因素加剧了肌营养不良蛋白的缺乏。这种额外的因素可能与质膜病变有关（mdx中未见）。或者，小鼠中可能存在补偿肌营养不良蛋白缺乏的因子。为了更好地理解mdx和DMD中发生的不同过程，我们需要更多地了解控制正常和病理人类和小鼠肌肉中肌肉生长和结缔组织生长之间平衡的因素。
Ultrastructurally there are some clear differences in the pathology of muscle in X chromosome-linked muscular dystrophy of the mouse (mdx) and Duchenne muscular dystrophy (DMD). In particular the mouse muscle does not become infiltrated by lare aggregations of connective tissue. It has been proposed that the differences are due to secondary biochemical changes consequent on the absence of dystrophin in both conditions. If this is the case, attention should be directed to the earliest ultrastructural changes held in common by both disorders. The most conspicuous of these, preceding myofibril breakdown, is dilation of the sarcoplasmic reticulum. Any physiological link between this and the absence of dystrophin remains to be determined. We suggest that in the mdx mouse, the widespread myofibre necrosis occurring at 3-4 weeks is triggered by increased mechanical demands causing the lack of dystrophin to become critical at this time. Subsequent regeneration of the myofibres appears to be almost completely successful. The ultimate failure of regeneration in DMD, in contrast, may be due to an additional factors acting in DMD exacerbating the lack of dystrophin. This additonal factor may be associated with the plasma membrane lesions (not seen in mdx). Alternatively there may be factors present in the mouse that compensate for the lack of dystrophin. It is pointed out that to understand better the different processes ocurring in mdx and DMD we need to learn more about the factors which control the balance between the growth of muscle and the growth of connective tissue in both normal and pathological human and mouse muscle.