中国神经再生研究(英文版) ›› 2020, Vol. 15 ›› Issue (4): 636-645.doi: 10.4103/1673-5374.266903

• 综述:退行性病与再生 • 上一篇    下一篇

髓鞘疾病中未折叠蛋白应答

  

  • 出版日期:2020-04-15 发布日期:2020-05-28

Unfolded protein response in myelin disorders

Wensheng Lin1, 2, Sarrabeth Stone1, 2   

  1. 1 Department of Neuroscience, University of Minnesota, Minneapolis, MN, USA
    2 Institute for Translational Neuroscience, University of Minnesota, Minneapolis, MN, USA
  • Online:2020-04-15 Published:2020-05-28
  • Contact: Wensheng Lin, MD, PhD,linw@umn.edu.
  • Supported by:
    This work was supported by grants from the National Institutes of Health (NS094151 and NS105689, both to WL).

摘要: orcid: 0000-0003-4931-7642 (Wensheng Lin)

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.

Key words: axon, ER, multiples sclerosis, myelin, oligodendrocyte, Schwann cell, spinal cord injury, UPR