THE MDX MOUSE SKELETAL-MUSCLE MYOPATHY .1. A HISTOLOGICAL, MORPHOMETRIC AND BIOCHEMICAL INVESTIGATION
THE MDX MOUSE SKELETAL-MUSCLE MYOPATHY .1. A HISTOLOGICAL, MORPHOMETRIC AND BIOCHEMICAL INVESTIGATION
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DOI：
10.1111/j.1365-2990.1988.tb00866.x
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发表时间：
1988-01-01
影响因子：
5
通讯作者：
SLOPER, JC
中科院分区：
文献类型：
--
作者：
COULTON, GR;MORGAN, JE;SLOPER, JC
已对肌病小鼠 mdx 品系的骨骼肌进行了检查。 65 只 22 至 303 天龄的小鼠表现出广泛且反复发生的坏死和肌纤维再生区域，通常伴有活跃的细胞浸润。比目鱼肌的形态测量显示小肌纤维和大肌纤维的比例异常；超过一半的肌纤维含有“中央”（非外周）细胞核。未检测到组织化学肌纤维类型分组。所有动物中肌肉来源酶的血清活性均大大升高，这可能反映了受损肌肉纤维中酶的渗漏。在 13 只小鼠中发现了心肌病的组织学证据。因此，mdx 肌病表现出杜氏肌营养不良症的特征。 Mdx 与杜氏营养不良的主要不同之处在于，它表现出更大程度的代偿性肌肉再生，并且不存在肌肉纤维的纤维脂肪替代。
Skeletal muscle has been examined in a colony of the mdx strain of myopathic mice. Sixty-five mice from 22 to 303 days of age, showed extensive and recurrent areas of necrosis and regeneration of muscle fibres, often accompanied by active cellular infiltration. Morphometry of the soleus muscle revealed an abnormal proportion of small and large muscle fibres; over half of the muscle fibres contained ''central'' (nonperipheral) nuclei. No histochemical muscle fibre-type grouping was detected. Serum activities of muscle-derived enzymes were greatly elevated in all animals and probably reflect enzyme leakage from damaged muscle fibres. Histological evidence of a cardiomyopathy was found in 13 mice. The mdx myopathy thus shows features seen in Duchenne muscular dystrophy. Mdx differs from Duchenne dystrophy principally in that it exhibits a greater degree of compensatory muscle regeneration and an absence of fibro-fatty replacement of muscle fibres.