Journal
NEUROMUSCULAR DISORDERS
Volume 22, Issue 5, Pages 406-417Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2011.10.011
Keywords
Duchenne muscular dystrophy; Dystrophin; Utrophin; Functional tests; Histopathology; Biomarkers
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Funding
- FP6 [036825]
- FP7 BIO-NMD Project [241665]
- Dutch Organization for Scientific Research (NWO/ZonMW)
- Dutch Duchenne Parent Project
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The genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/-) are more severely affected than mdx mice, but outlive mdx/utrn -/- mice. We subjected mdx/utrn +/+, +/-, -/- and wild type males to a 12 week functional test regime of four different functional tests. Mdx/utrn +/+ and +/- mice completed the regime, while mdx/utrn -/- mice died prematurely. Mdx/utrn +/- mice performed significantly worse compared to mdx/utrn +/+ mice in functional tests. Creatine kinase levels, percentage of fibrotic/necrotic tissue, morphology of neuromuscular synapses and expression of biomarker genes were comparable, whereas mdx/utrn +/- and -/- mice had increased levels of regenerating fibers. This makes mdx/utrn +/- mice valuable for testing the benefit of potential therapies on muscle function parameters. (C) 2011 Elsevier B.V. All rights reserved.
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