A marginal level of dystrophin partially ameliorates hindlimb muscle passive mechanical properties in dystrophin-null mice
A marginal level of dystrophin partially ameliorates hindlimb muscle passive mechanical properties in dystrophin-null mice
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DOI：
10.1002/mus.23536
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发表时间：
2012-12-01
期刊：
影响因子：
3.4
通讯作者：
Duan, Dongsheng
中科院分区：
文献类型：
--
作者：
Hakim, Chady H.;Duan, Dongsheng
本研究的目的是确定最低水平的肌营养不良蛋白表达是否能改善小鼠杜氏肌营养不良模型骨骼肌的被动力学性能。方法：比较mdx3cv和mdx4cv小鼠6、14和20月龄时指长伸肌（EDL）的弹性和粘性特性。这两种菌株都在C57Bl/6背景上，都失去了全长的肌营养不良蛋白。有趣的是，mdx3cv小鼠在？正常水平的5%。结果：与年龄匹配的mdx4cv小鼠相比，我们发现mdx3cv小鼠各年龄组EDL的应力应变谱和应力松弛率都得到了部分保留。结论：低水平的肌营养不良蛋白表达可能与杜氏肌营养不良患者的肌肉僵硬有关。中国机械工程学报（英文版），2012
Introduction: The goal of this study was to determine whether a minimal level of dystrophin expression improves the passive mechanical properties of skeletal muscle in the murine Duchenne muscular dystrophy model. Methods: We compared the elastic and viscous properties of the extensor digitorum longus muscle (EDL) in mdx3cv and mdx4cv mice at 6, 14, and 20 months of age. Both strains are on the C57Bl/6 background, and both lose the full-length dystrophin protein. Interestingly, mdx3cv mice express a near full-length dystrophin at ?5% of the normal level. Results: We found that the stressstrain profile and the stress relaxation rate of the EDL in mdx3cv mice were partially preserved in all age groups compared with age-matched mdx4cv mice. Conclusion: Our results suggest that a low level of dystrophin expression may treat muscle stiffness in Duchenne muscular dystrophy. Muscle Nerve 46: 948-950, 2012