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Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice
P0 glycoprotein is an abundant product of terminal differentiation in myelinating Schwann cells. The mutant P0S63del causes Charcot-Marie-Tooth 1B neuropathy in humans, and a very similar demyelinating neuropathy in transgenic mice. P0S63del is retained in the endoplasmic reticulum of Schwann cells,...
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| Hauptverfasser: | , , , , , , , |
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| Format: | Artigo |
| Sprache: | Inglês |
| Veröffentlicht: |
The Rockefeller University Press
2013
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| Schlagworte: | |
| Online Zugang: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3620355/ https://ncbi.nlm.nih.gov/pubmed/23547100 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1084/jem.20122005 |
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