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

@article{Putten2012ComparisonOS,
  title={Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains},
  author={Maaike van Putten and Darshan Kumar and Margriet Hulsker and Willem M.H. Hoogaars and Jaap J. Plomp and A. John van Opstal and Maarten van Iterson and Peter JJ. Admiraal and Gert-Jan B. van Ommen and Peter A.C. ‘t Hoen and Annemieke Aartsma-Rus},
  journal={Neuromuscular Disorders},
  year={2012},
  volume={22},
  pages={406-417}
}
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… CONTINUE READING
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