Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle.
Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle.
复制标题
DOI：
10.1083/jcb.200210117
复制
发表时间：
2003-05-12
期刊：
影响因子：
--
通讯作者：
Melki J
中科院分区：
文献类型：
--
作者：
Nicole S;Desforges B;Millet G;Lesbordes J;Cifuentes-Diaz C;Vertes D;Cao ML;De Backer F;Languille L;Roblot N;Joshi V;Gillis JM;Melki J
缺失的小鼠Smn外显子7，最常见的突变发现脊髓性肌萎缩症，已被定向到卫星细胞，肌肉祖细胞和融合的肌管，或融合的肌管。当卫星细胞发生突变时，突变小鼠发生严重的肌病过程，进行性运动麻痹，并在1月龄时过早死亡（严重突变）。严重突变体的肌肉再生障碍与成肌前体细胞的缺陷有关。相比之下，当卫星细胞保持完整时，突变小鼠发生类似的肌病过程，但表现出轻度表型，中位生存期为8个月，运动表现与对照组相似（轻度突变）。在轻度突变体中观察到高比例的表达SMN的再生肌纤维，以补偿前6个月年龄内成熟肌纤维的进行性丢失。然后，尽管肌纤维具有正常的收缩特性，但轻度突变体会发展为肌肉力量和质量的减少。肌肉再生过程的进行性衰退不再能够抵消肌肉变性，导致肌纤维的急剧损失。这些数据表明，完整的卫星细胞显着提高慢性肌病突变小鼠的生存和运动性能，并建议卫星细胞再生骨骼肌的潜力有限。
Deletion of murine Smn exon 7, the most frequent mutation found in spinal muscular atrophy, has been directed to either both satellite cells, the muscle progenitor cells and fused myotubes, or fused myotubes only. When satellite cells were mutated, mutant mice develop severe myopathic process, progressive motor paralysis, and early death at 1 mo of age (severe mutant). Impaired muscle regeneration of severe mutants correlated with defect of myogenic precursor cells both in vitro and in vivo. In contrast, when satellite cells remained intact, mutant mice develop similar myopathic process but exhibit mild phenotype with median survival of 8 mo and motor performance similar to that of controls (mild mutant). High proportion of regenerating myofibers expressing SMN was observed in mild mutants compensating for progressive loss of mature myofibers within the first 6 mo of age. Then, in spite of normal contractile properties of myofibers, mild mutants develop reduction of muscle force and mass. Progressive decline of muscle regeneration process was no more able to counterbalance muscle degeneration leading to dramatic loss of myofibers. These data indicate that intact satellite cells remarkably improve the survival and motor performance of mutant mice suffering from chronic myopathy, and suggest a limited potential of satellite cells to regenerate skeletal muscle.
登录
查看更多内容
影响因子：
7.8
作者：
Asakura, Atsushi;Seale, Patrick;Girgis-Gabardo, Adele;Rudnicki, Michael A
通讯作者：
Rudnicki, Michael A
DOI：
10.1083/jcb.200108150
发表时间：
2002-05-27
期刊：
The Journal of cell biology
影响因子：
--
作者：
Qu-Petersen Z;Deasy B;Jankowski R;Ikezawa M;Cummins J;Pruchnic R;Mytinger J;Cao B;Gates C;Wernig A;Huard J
通讯作者：
Huard J
影响因子：
15.9
作者：
Cossu, G;Mavilio, F
通讯作者：
Mavilio, F
影响因子：
7.5
作者：
Paushkin, S;Gubitz, AK;Dreyfuss, G
通讯作者：
Dreyfuss, G
影响因子：
3.5
作者：
Burlet, P;Huber, C;Lefebvre, S
通讯作者：
Lefebvre, S