Pastoret C, Sebille A
Laboratoire de Physiologie, Atelier de Régénération Neuromusculaire, Faculté de Médecine Saint-Antoine, Paris, France.
Histochemistry. 1993 Oct;100(4):271-6. doi: 10.1007/BF00270046.
The mdx mutant is a murine homologous model of Duchenne muscular dystrophy (DMD). Fibre types determined by the myosin-ATPase technique in soleus muscles were compared in C57BL/10 control and mdx mice from 3 to 52 weeks of age. The control strain continuously presented 70% of type 2 fibres whereas the mdx strain showed an increase to 80% at 6 weeks and a subsequent decline. In mdx muscles, necrosis which begins at 3 weeks of age did not affect specifically one type of fibre. Type grouping was never observed when muscle regeneration occurred. Fibres of intermediate type (1C and 2C) were found continuously up to 52 weeks of age in the mdx mutant. The foci of small immature regenerating fibres were of type 2C but never 1C. A few mature fibres were either of type 2C or 1C. We suggest that the presence of intermediate type fibres could result from the co-expression of type 1 and 2 myosin heavy chains, indicating a transition from type 2 to type 1 in regenerating fibres.
mdx突变体是杜兴氏肌营养不良症(DMD)的小鼠同源模型。采用肌球蛋白-ATP酶技术,对3至52周龄的C57BL/10对照小鼠和mdx小鼠比目鱼肌中的纤维类型进行了比较。对照品系中2型纤维持续占70%,而mdx品系在6周龄时增加到80%,随后下降。在mdx小鼠的肌肉中,3周龄开始出现的坏死并未特异性影响某一种纤维类型。肌肉再生时从未观察到纤维分组现象。在mdx突变体中,中间型(1C和2C)纤维一直存在至52周龄。小的未成熟再生纤维灶为2C型,但从未出现过1C型。少数成熟纤维要么是2C型,要么是1C型。我们认为,中间型纤维的存在可能是由于1型和2型肌球蛋白重链的共表达所致,这表明再生纤维正从2型向1型转变。