ABLATION OF THE UPR–MEDIATOR CHOP RESTORES MOTOR FUNCTION AND REDUCES DEMYELINATION IN CHARCOT MARIE TOOTH 1B MICE

Deletion of serine 63 from P0 glycoprotein (P0S63del) causes Charcot-Marie-Tooth 1B neuropathy in humans, and P0S63del produces a very similar demyelinating neuropathy in transgenic mice. P0S63del is retained in the endoplasmic reticulum and fails to be incorporated into myelin. Here we report that...

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Main Authors: Pennuto, Maria, Tinelli, Elisa, Malaguti, MariaChiara, Del Carro, Ubaldo, D'Antonio, Maurizio, Ron, David, Quattrini, Angelo, Feltri, M. Laura, Wrabetz, Lawrence
Format: Artigo
Sprog:Inglês
Udgivet: 2008
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2267889/
https://ncbi.nlm.nih.gov/pubmed/18255032
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.neuron.2007.12.021
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