Endoplasmic reticulum stress in disorders of myelinating cells

Wensheng Lin*, Brian Popko

*Corresponding author for this work

Research output: Contribution to journalReview article

158 Scopus citations

Abstract

Myelinating cells, oligodendrocytes in the CNS and Schwann cells in the peripheral nervous system produce an enormous amount of plasma membrane during the myelination process, making them particularly susceptible to disruptions of the secretory pathway. Endoplasmic reticulum stress, initiated by the accumulation of unfolded or misfolded proteins, activates the unfolded protein response, which adapts cells to the stress. If this adaptive response is insufficient, the unfolded protein response activates an apoptotic program to eliminate the affected cells. Recent observations suggest that endoplasmic reticulum stress in myelinating cells is important in the pathogenesis of various disorders of myelin, including Charcot-Marie-Tooth disease, Pelizaeus-Merzbacher disease and Vanishing White Matter Disease, as well as in the most common myelin disorder, multiple sclerosis. A better understanding of endoplasmic reticulum stress in myelinating cells has laid the groundwork for the design of new therapeutic strategies for promoting myelinating cell survival in these disorders.

Original languageEnglish (US)
Pages (from-to)379-385
Number of pages7
JournalNature Neuroscience
Volume12
Issue number4
DOIs
StatePublished - Apr 1 2009

ASJC Scopus subject areas

  • Neuroscience(all)

Fingerprint Dive into the research topics of 'Endoplasmic reticulum stress in disorders of myelinating cells'. Together they form a unique fingerprint.

Cite this