Article
Activation of the MEF2 transcription factor in skeletal muscles from myotonic mice.
The Journal of clinical investigation - 1 May 2002
Wu Hai, Olson Eric N
Abstract excerpt
Becker syndrome, a recessive nondystrophic myotonia caused by mutations in the chloride channel 1 gene (CLCN1), is characterized by delayed muscle relaxation after contraction. The ADR (arrested development of righting response) mouse is an animal model for Becker syndrome. Skeletal muscles from ADR myotonic animals show an increased number of oxidative fibers with a lack of glycolytic fibers as well as signs of...
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