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Or Kakhlon, Hava Glickstein, Naomi Feinstein, Yan Liu, Otto Baba, Tatsuo Terashima, Hasan Orhan Akman, Salvatore DiMauro and Alexander Lossos Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase Journal of Neurochemistry 127

Version of Record online: 9 MAY 2013 | DOI: 10.1111/jnc.12277

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Knockdown of glycogen branching enzyme in neurons led to accumulation of an insoluble form of glycogen called polyglucosan, to apoptosis and to activation of glycogen synthase. These effects were reversed by glycogen synthase inhibition through starvation and rapamycin treatments, suggesting a potential therapeutic value of glycogen synthase inhibition for treating glycogen storage disorders.

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