Department of Human Genetics, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.
Neuromuscul Disord. 2010 Apr;20(4):273-80. doi: 10.1016/j.nmd.2010.02.004. Epub 2010 Mar 21.
To assess the effect of potential therapeutic agents in dystrophic mice it is useful to have a functional test regime that does not affect the natural disease progression of mdx mice with dystrophinopathy. We determined the effect of a 12 week test regime consisting of fore limb grip strength, rotarod analysis and two and four limb hanging wire tests on the disease progression of 4-week-old mdx mice. Mice performed the different functional tests on consecutive days on a weekly basis. No difference was found in serum creatine kinase levels between functionally active and sedentary mice. The percentage of fibrotic/necrotic areas assessed in a semi-automated way with colour deconvolution of skeletal muscles, heart and diaphragm did not vary within muscles or between groups, nor did the gene expression levels of disease-related genes. We conclude that this test regime may be suitable for short-term functional evaluation of therapeutic approaches in the mdx mouse.
为了评估潜在治疗药物在肌营养不良症小鼠中的效果，最好有一种不会影响肌营养不良症 mdx 小鼠自然疾病进展的功能测试方案。我们确定了一项为期 12 周的测试方案的效果，该方案包括前肢握力、旋转轮分析以及双前肢和四后肢悬挂线测试，以评估 4 周龄 mdx 小鼠的疾病进展情况。小鼠每周连续几天进行不同的功能测试。在功能活跃和久坐不动的小鼠之间，血清肌酸激酶水平没有差异。使用骨骼肌、心脏和膈肌的颜色反卷积以半自动方式评估的纤维化/坏死区域在肌肉内或组间没有变化，疾病相关基因的基因表达水平也没有变化。我们得出结论，该测试方案可能适用于 mdx 小鼠中短期治疗方法的功能评估。
