Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation
Abstract
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 reticulum (ER) stress pathways, but poor PLP1 genotype-phenotype associations suggest additional pathogenetic mechanisms. Using induced pluripotent...
Paper Details
Title
Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation
Published Date
Oct 1, 2019
Journal
Volume
25
Issue
4
Pages
531 - 541.e6
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Notes
History