Weller B, Karpati G, Lehnert S, Carpenter S, Ajdukovic B, Holland P
Neuromuscular Research Group, Montreal Neurological Institute, Quebec, Canada.
Am J Pathol. 1991 Jun;138(6):1497-502.
In skeletal muscles of young mdx female heterozygote mice, there is a mosaic of dystrophin-positive and dystrophin-negative fiber segments. In older animals, there is a marked decline in the number of dystrophin-negative fiber segments. This phenomenon might be due to a fusion of dystrophin-competent satellite cells into the originally dystrophin-negative fiber segments during growth. To study this possibility, soleus muscles of 10-day-old mdx female heterozygotes were gamma irradiated (2000 rads) to inhibit subsequent myosatellite cell proliferation and fusion. In the irradiated soleus muscles of animals at 60 days, the relative amount of dystrophin measured by quantitative immunoblots was not significantly different from that of the contralateral nonirradiated muscles. The prevalence of dystrophin-negative fibers in the 60-day-old irradiated solei was not higher than in the nonirradiated contralateral muscles, implying that dystrophin-competent satellite cell fusion was not a significant factor in the observed conversion. A longitudinal expansion of the cytoplasmic domain of the original dystrophin-competent myonuclei during growth could explain the observed conversion phenomenon.
在年轻的mdx雌性杂合子小鼠的骨骼肌中,存在抗肌萎缩蛋白阳性和抗肌萎缩蛋白阴性纤维节段的镶嵌现象。在年龄较大的动物中,抗肌萎缩蛋白阴性纤维节段的数量显著下降。这种现象可能是由于在生长过程中,具有产生抗肌萎缩蛋白能力的卫星细胞融合到原本抗肌萎缩蛋白阴性的纤维节段中。为了研究这种可能性,对10日龄的mdx雌性杂合子的比目鱼肌进行γ射线照射(2000拉德),以抑制随后的肌卫星细胞增殖和融合。在60日龄动物经照射的比目鱼肌中,通过定量免疫印迹法测得的抗肌萎缩蛋白的相对含量与对侧未照射肌肉的抗肌萎缩蛋白相对含量没有显著差异。60日龄经照射的比目鱼肌中抗肌萎缩蛋白阴性纤维的比例并不高于未照射的对侧肌肉,这意味着具有产生抗肌萎缩蛋白能力的卫星细胞融合不是观察到的转化现象的重要因素。在生长过程中,原本具有产生抗肌萎缩蛋白能力的肌细胞核的细胞质结构域纵向扩展可以解释观察到的转化现象。