Chen Songlin, Zhang Cheng, Liu Xiaorong, Gao Ling, Zhang Weixi, Huang Wen, Lu Xilin, Wang Zhanhang
Department of Neurology, First Affiliated Hospital of Zhong Shan University, Guangzhou 510080, China.
Sichuan Da Xue Xue Bao Yi Xue Ban. 2003 Apr;34(2):210-3.
With a view to further experimental studies on Duchenne muscular dystrophy (DMD), we investigated the motor function, serum creatine kinase (CK) level and pathological characteristics of muscular tissue in C57, mdx, dystrophin/utrophin gene double knockout (dko)mice.
Gene identification was performed for the filial generation of heterozygote of utrophin gene knockout of mdx mice for acquiring mdx and dko mice. The motor function, serum CK level, and the pathology of muscular tissue of C57, mdx and dko mice were compared.
There were significant differences in all detecting aspects between C57 mouse and dko mouse. In the aspects of serum CK level and pathology of muscular tissue, there were differences between C57 mouse and mdx mouse; however, no significant difference in motor function was observed between them.
Compared with mdx mouse, dko mouse is in a more serious illness state which is nearer to the natural state of DMD. Therefore, dko mouse is a more ideal model for studying the praxiology of DMD.
为了进一步开展杜氏肌营养不良症（DMD）的实验研究，我们对C57、mdx、抗肌萎缩蛋白/肌营养不良蛋白基因双敲除（dko）小鼠的运动功能、血清肌酸激酶（CK）水平及肌肉组织病理特征进行了研究。
对mdx小鼠肌营养不良蛋白基因敲除杂合子的子代进行基因鉴定，以获得mdx和dko小鼠。比较C57、mdx和dko小鼠的运动功能、血清CK水平及肌肉组织病理学。
C57小鼠与dko小鼠在所有检测方面均存在显著差异。在血清CK水平和肌肉组织病理学方面，C57小鼠与mdx小鼠存在差异；然而，它们在运动功能方面未观察到显著差异。
与mdx小鼠相比，dko小鼠病情更严重，更接近DMD的自然状态。因此，dko小鼠是研究DMD行为学更理想的模型。
