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Structural and functional alterations of muscle fibres in the novel mouse model of facioscapulohumeral muscular dystrophy

We recently generated a mouse model of facioscapulohumeral muscular dystrophy (FSHD) by selectively overexpressing FRG1, a candidate gene for FSHD, in skeletal muscle. The muscles of the FRG-1 mice did not show any plasmamembrane defect suggesting a novel pathogenetic mechanism for FSHD. Here, we st...

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Autors principals: D'Antona, Giuseppe, Brocca, Lorenza, Pansarasa, Orietta, Rinaldi, Chiara, Tupler, Rossella, Bottinelli, Roberto
Format: Artigo
Idioma:Inglês
Publicat: Blackwell Science Inc 2007
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2277004/
https://ncbi.nlm.nih.gov/pubmed/17855756
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1113/jphysiol.2007.141481
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