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

Hiroko Nobuta, Nan Yang, Yi Han Ng, Samuele G. Marro, Khalida Sabeur, Manideep Chavali, John H. Stockley, David W. Killilea, Patrick B. Walter, Chao Zhao, Philip Huie, Steven A. Goldman, Arnold R. Kriegstein, Robin J.M. Franklin, David H. Rowitch, Marius Wernig

Research output: Contribution to journalArticlepeer-review

27 Scopus citations

Abstract

Pelizaeus-Merzbacher disease is a pediatric leukodystrophy causing oligodendrocyte cell death. Nobuta et al. show that mutations in human PLP1 gene cause iron-induced cell death through lipid peroxidation, abnormal iron metabolism, and hypersensitivity to free iron. Iron chelation rescues cell death, offering a therapeutic direction for a disease without current treatments.

Original languageEnglish (US)
Pages (from-to)531-541.e6
JournalCell Stem Cell
Volume25
Issue number4
DOIs
StatePublished - Oct 3 2019
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Molecular Medicine
  • Genetics
  • Cell Biology

Keywords

  • ferroptosis
  • gene correction
  • induced pluripotent stem cells
  • iron chelation
  • leukodystrophy
  • myelination
  • oligodendrocyte
  • patient models

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