Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation

Pelizaeus-Merzbacher disease (PMD) is an X-linked leukodystrophy caused by mutations in Proteolipid Protein 1 (PLP1), encoding a major myelin protein, resulting in profound developmental delay and early lethality. Previous work showed involvement of unfolded protein response (UPR) and endoplasmic re...

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Published in:Cell Stem Cell
Main Authors: Nobuta, Hiroko, Yang, Nan, Ng, Yi Han, Marro, Samuele G., Sabeur, Khalida, Chavali, Manideep, Stockley, John H., Killilea, David W., Walter, Patrick B., Zhao, Chao, Huie, Philip, Goldman, Steven A., Kriegstein, Arnold R., Franklin, Robin J.M., Rowitch, David H., Wernig, Marius
Format: Artigo
Language:Inglês
Published: 2019
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC8282124/
https://ncbi.nlm.nih.gov/pubmed/31585094
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.stem.2019.09.003
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