A 3 months mild functional test regime does not affect disease parameters in young mdx mice

作者：van Putten Maaike; de Winter Christa; van Roon Mom Willeke; van Ommen Gert Jan; 't Hoen Peter A C; Aartsma Rus Annemieke^*

来源：Neuromuscular Disorders, 2010, 20(4): 273-280.

DOI：10.1016/j.nmd.2010.02.004

• Mdx mouse
• Duchenne Muscular Dystrophy
• Histopathology
• Biomarkers
• Functional tests

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.

• 出版日期2010-4