Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase.

@article{Kakhlon2013PolyglucosanNC,
  title={Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase.},
  author={Or Kakhlon and Hava Glickstein and Naomi Feinstein and Yan Liu and Otto Baba and Tatsuo Terashima and Hasan Orhan Akman and Salvatore Dimauro and Alexander Lossos},
  journal={Journal of neurochemistry},
  year={2013},
  volume={127 1},
  pages={101-13}
}
Uncontrolled elongation of glycogen chains, not adequately balanced by their branching, leads to the formation of an insoluble, presumably neurotoxic, form of glycogen called polyglucosan. To test the suspected pathogenicity of polyglucosans in neurological glycogenoses, we have modeled the typical glycogenosis Adult Polyglucosan Body Disease (APBD) by suppressing glycogen branching enzyme 1 (GBE1, EC 2.4.1.18) expression using lentiviruses harboring short hairpin RNA (shRNA). GBE1 suppression… CONTINUE READING

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