A maladaptive ER stress response triggers dysfunction in highly active muscles of mice with SELENON loss
Selenoprotein N (SELENON) is an endoplasmic reticulum (ER) protein whose loss of function leads to human SELENON-related myopathies.SelenoN knockout Dog Apparel (KO) mouse limb muscles, however, are protected from the disease, and display no major alterations in muscle histology or contractile properties.Interestingly, we find that the highly activ