Journal
NEUROMUSCULAR DISORDERS
Volume 20, Issue 4, Pages 273-280Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2010.02.004
Keywords
Mdx mouse; Duchenne Muscular Dystrophy; Histopathology; Biomarkers; Functional tests
Categories
Funding
- TREAT-NMD network of excellence
- ZonNW
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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. (C) 2010 Elsevier B.V. All rights reserved.
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