Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains

Maaike van Putten, Darshan Kumar, Margriet Hulsker, Willem M H Hoogaars, Jaap J Plomp, Annemarieke van Opstal, Maarten van Iterson, Peter Admiraal, Gert-Jan B van Ommen, Peter A C 't Hoen, Annemieke Aartsma-Rus
Neuromuscular Disorders: NMD 2012, 22 (5): 406-17
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 12week 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.

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