Unfolded protein response in myelin disorders

Wensheng Lin, Sarrabeth Stone

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations

Abstract

Activation of the unfolded protein response in response to endoplasmic reticulum stress preserves cell viability and function under stressful conditions. Nevertheless, persistent, unresolvable activation of the unfolded protein response can trigger apoptosis to eliminate stressed cells. Recent studies show that the unfolded protein response plays an important role in the pathogenesis of various disorders of myelin, including multiples sclerosis, Charcot-Marie-Tooth disease, Pelizaeus-Merzbacher disease, vanishing white matter disease, spinal cord injury, tuberous sclerosis complex, and hypoxia-induced perinatal white matter injury. In this review we summarize the current literature on the unfolded protein response and the evidence for its role in the pathogenesis of myelin disorders.

Original languageEnglish (US)
Pages (from-to)636-645
Number of pages10
JournalNeural Regeneration Research
Volume15
Issue number4
DOIs
StatePublished - Apr 1 2020

Bibliographical note

Funding Information:
This work was supported by grants from the National Institutes of Health (NS094151 and NS105689, both to WL).

Keywords

  • ER
  • Schwann cell
  • UPR
  • axon
  • multiples sclerosis
  • myelin
  • oligodendrocyte
  • spinal cord injury

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