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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Veröffentlicht in:Cell Stem Cell
Hauptverfasser: 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
Sprache:Inglês
Veröffentlicht: 2019
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Article
Online Zugang: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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