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

doi:10.1016/j.stem.2019.09.003

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 journal › Article › peer-review

100 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 language	English
Pages (from-to)	531-541.e6
Journal	Cell Stem Cell
Volume	25
Issue number	4
DOIs	https://doi.org/10.1016/j.stem.2019.09.003
State	Published - 3 Oct 2019
Externally published	Yes

Keywords

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

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10.1016/j.stem.2019.09.003

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Nobuta, H., Yang, N., Ng, Y. H., Marro, S. G., Sabeur, K., Chavali, M., Stockley, J. H., Killilea, D. W., Walter, P. B., Zhao, C., Huie, P., Goldman, S. A., Kriegstein, A. R., Franklin, R. J. M., Rowitch, D. H., & Wernig, M. (2019). Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation. Cell Stem Cell, 25(4), 531-541.e6. https://doi.org/10.1016/j.stem.2019.09.003

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title = "Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation",

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.",

keywords = "ferroptosis, gene correction, induced pluripotent stem cells, iron chelation, leukodystrophy, myelination, oligodendrocyte, patient models",

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

note = "Publisher Copyright: {\textcopyright} 2019",

year = "2019",

month = oct,

day = "3",

doi = "10.1016/j.stem.2019.09.003",

language = "English",

volume = "25",

pages = "531--541.e6",

journal = "Cell Stem Cell",

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publisher = "Cell Press",

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Nobuta, H, Yang, N, Ng, YH, Marro, SG, Sabeur, K, Chavali, M, Stockley, JH, Killilea, DW, Walter, PB, Zhao, C, Huie, P, Goldman, SA, Kriegstein, AR, Franklin, RJM, Rowitch, DH & Wernig, M 2019, 'Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation', Cell Stem Cell, vol. 25, no. 4, pp. 531-541.e6. https://doi.org/10.1016/j.stem.2019.09.003

TY - JOUR

T1 - Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron Chelation

AU - Nobuta, Hiroko

AU - Yang, Nan

AU - Ng, Yi Han

AU - Marro, Samuele G.

AU - Sabeur, Khalida

AU - Chavali, Manideep

AU - Stockley, John H.

AU - Killilea, David W.

AU - Walter, Patrick B.

AU - Zhao, Chao

AU - Huie, Philip

AU - Goldman, Steven A.

AU - Kriegstein, Arnold R.

AU - Franklin, Robin J.M.

AU - Rowitch, David H.

AU - Wernig, Marius

PY - 2019/10/3

Y1 - 2019/10/3

N2 - 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.

AB - 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.

KW - ferroptosis

KW - gene correction

KW - induced pluripotent stem cells

KW - iron chelation

KW - leukodystrophy

KW - myelination

KW - oligodendrocyte

KW - patient models

UR - https://www.scopus.com/pages/publications/85072631267

U2 - 10.1016/j.stem.2019.09.003

DO - 10.1016/j.stem.2019.09.003

M3 - Article

C2 - 31585094

AN - SCOPUS:85072631267

SN - 1934-5909

VL - 25

SP - 531-541.e6

JO - Cell Stem Cell

JF - Cell Stem Cell

IS - 4

ER -

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

Abstract

Keywords

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