Article
Mutation types and aging differently affect revertant fiber expansion in dystrophic mdx and mdx52 mice.
PloS one - 1 Jan 2013
Echigoya Yusuke, Lee Joshua, Rodrigues Merryl, Nagata Tetsuya, Tanihata Jun, Nozohourmehrabad Ashkan, Panesar Dharminder, Miskew Bailey, Aoki Yoshitsugu, Yokota Toshifumi
Abstract excerpt
Duchenne muscular dystrophy (DMD), one of the most common and lethal genetic disorders, and the mdx mouse myopathies are caused by a lack of dystrophin protein. These dystrophic muscles contain sporadic clusters of dystrophin-expressing revertant fibers (RFs), as detected by immunohistochemistry. RFs are known to arise from muscle precursor cells with spontaneous exon skipping (alternative splicing) and clonally...
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