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Article

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

14 Sept 2007

Abstract excerpt

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 study structure and function of muscle fibres from three lines of mice overexpressing FRG1 at different levels:...

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PMID: 17855756
DOI: 10.1113/jphysiol.2007.141481

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