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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Publicat a:Cell Stem Cell
Autors principals: 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
Idioma:Inglês
Publicat: 2019
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Article
Accés en línia: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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