Article
Mechanism suppressing glycogen synthesis in neurons and its demise in progressive myoclonus epilepsy.
Nature neuroscience - 1 Nov 2007
Vilchez David, Ros Susana, Cifuentes Daniel, Pujadas Lluís, Vallès Jordi, García-Fojeda Belén, Criado-García Olga, Fernández-Sánchez Elena, Medraño-Fernández Iria, Domínguez Jorge, García-Rocha Mar, Soriano Eduardo, Rodríguez de Córdoba Santiago, Guinovart Joan J
Abstract excerpt
Glycogen synthesis is normally absent in neurons. However, inclusion bodies resembling abnormal glycogen accumulate in several neurological diseases, particularly in progressive myoclonus epilepsy or Lafora disease. We show here that mouse neurons have the enzymatic machinery for synthesizing glycogen, but that it is suppressed by retention of muscle glycogen synthase (MGS) in the phosphorylated, inactive state....
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