Article
Increased muscle stress-sensitivity induced by selenoprotein N inactivation in mouse: a mammalian model for SEPN1-related myopathy.
PloS one - 1 Jan 2011
Rederstorff Mathieu, Castets Perrine, Arbogast Sandrine, Lainé Jeanne, Vassilopoulos Stéphane, Beuvin Maud, Dubourg Odile, Vignaud Alban, Ferry Arnaud, Krol Alain, Allamand Valérie, Guicheney Pascale, Ferreiro Ana, Lescure Alain
Abstract excerpt
Selenium is an essential trace element and selenoprotein N (SelN) was the first selenium-containing protein shown to be directly involved in human inherited diseases. Mutations in the SEPN1 gene, encoding SelN, cause a group of muscular disorders characterized by predominant affection of axial muscles. SelN has been shown to participate in calcium and redox homeostasis, but its pathophysiological role in skeletal...
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