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Primary sodium storage disease (NSSD) is a severe neonatal disease caused by mutations in either E1 or E2 subunits of the ATPase in humans and rats. Despite the presence of the disease in both species, studies have been lacking to examine the associated changes at the biochemical level. Here we report the quantitative and spectral analysis of the Na+-K+ ATPase protein in a rat transgenic model of this disorder. The specific activity of the Na+-K+ ATPase was significantly reduced in skeletal muscle (8 +/- 3% of wild-type levels) while the protein abundance was only 20% of wild-type. Western blot analyses indicated an increase in the protein degradation rate. These changes were not observed in the brain, heart or kidney, which in contrast exhibited levels of the Na+-K+ ATPase comparable to wild type. The decrease in Na+-K+ ATPase activity in skeletal muscle is consistent with the in vivo reduction in body weight and muscle mass observed in NSSD animals. The quantitative and spectral analyses provide a sensitive measure of the effects of disease on Na+-K+ ATPase in this rat model.Q:

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