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Longitudinal in vivo muscle function analysis of the DMSXL mouse model of myotonic dystrophy type 1.

Neuromuscul Disord

December 2013

Institut de Myologie, UPMC Univ. Paris 6 UM76, Inserm U974, CNRS UMR7215, F-75651 Paris Cedex 13, France. Electronic address:

Myotonic dystrophy is the most common adult muscle dystrophy. In view of emerging therapies, which use animal models as a proof of principle, the development of reliable outcome measures for in vivo longitudinal study of mouse skeletal muscle function is becoming crucial. To satisfy this need, we have developed a device to measure ankle dorsi- and plantarflexion torque in rodents.

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