Article
Phospholamban overexpression in mice causes a centronuclear myopathy-like phenotype.
Disease models & mechanisms - 1 Aug 2015
Fajardo Val A, Bombardier Eric, McMillan Elliott, Tran Khanh, Wadsworth Brennan J, Gamu Daniel, Hopf Andrew, Vigna Chris, Smith Ian C, Bellissimo Catherine, Michel Robin N, Tarnopolsky Mark A, Quadrilatero Joe, Tupling A Russell
Abstract excerpt
Centronuclear myopathy (CNM) is a congenital myopathy that is histopathologically characterized by centrally located nuclei, central aggregation of oxidative activity, and type I fiber predominance and hypotrophy. Here, we obtained commercially available mice overexpressing phospholamban (Pln(OE)), a well-known inhibitor of sarco(endo)plasmic reticulum Ca(2+)-ATPases (SERCAs), in their slow-twitch type I skeletal...
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