MUSCULAR-DYSTROPHY IN THE MDX MOUSE - HISTOPATHOLOGY OF THE SOLEUS AND EXTENSOR DIGITORUM LONGUS MUSCLES
MUSCULAR-DYSTROPHY IN THE MDX MOUSE - HISTOPATHOLOGY OF THE SOLEUS AND EXTENSOR DIGITORUM LONGUS MUSCLES
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DOI：
10.1016/0022-510x(87)90219-x
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发表时间：
1987-08-01
影响因子：
4.4
通讯作者：
SHOTTON, DM
中科院分区：
文献类型：
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作者：
CARNWATH, JW;SHOTTON, DM
我们使用光学显微镜组织形态计量学来量化mdx小鼠比目鱼肌和趾长伸肌（EDL）肌肉中肌营养不良引起的发育组织病理学变化。我们发现，这种X-连锁疾病表现出早期纤维坏死，具有侵入性细胞的病灶，受影响的纤维、透明纤维的聚集，并且在混合比目鱼肌中，1型纤维的比例进行性增加，mdx比目鱼肌含有58 ± 0.0000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000 26周时，1型纤维含量为5%，而27周时。+-。4%。这种增加不是由于2型纤维的萎缩或缓慢的轴突再神经支配。尽管在EDL中只有5%的所有原始纤维存活26周，但患病的mdx纤维连续且成功地被具有内部核的新纤维取代，受影响的小鼠因此避免了X连锁的人杜氏肌营养不良症和埃默里-德赖富斯肌营养不良症的终末期组织病理学和身体残疾特征。纯合子mdx小鼠的预期寿命与正常C57 BL/10小鼠相同，行为表现正常。因此，mdx小鼠是研究肌纤维变性和再生过程的极好哺乳动物模型。
We have used light microscopic histomorphometry to quantify the developmental histopathological changes induced by muscular dystrophy in the soleus and extensor digitorum longus (EDL) muscles of the mdx mouse. We find that this X-linked disease exhibits early fibre necrosis with foci of invasive cells, clustering of affected fibres, hyaline fibres, and, in the mixed soleus muscle, a progressive increase in the proportion of type 1 fibres, the mdx soleus containing 58 .+-. 5% type 1 fibres by 26 weeks, compared with 27 .+-. 4% in control C57BL/10 ScSn mice. This increase is not due to atrophy or slow axon reinnervation of type 2 fibres. Although only 5% of all original fibres survive by 26 weeks in the EDL, the diseased mdx fibres are continuously and successfully replaced by new fibres with internal nuclei, the affected mice thus avoiding the end-stage histopathology and physical disability characteristic of the X-linked human Duchenne and Emery-Dreifuss muscular dystrophies. Homozygous mdx mice share the life expectancy of normal C57BL/10 mice and appear behaviourly normal. The mdx mouse is therefore an excellent mammalian model in which to study the process of muscle fibre degeneration and regeneration.