Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, 611731, China.
Mol Neurobiol. 2024 Aug;61(8):5295-5307. doi: 10.1007/s12035-023-03905-8. Epub 2024 Jan 5.
Preterm white matter injury (WMI) is a demyelinating disease with high incidence and mortality in premature infants. Oligodendrocyte cells (OLs) are a specialized glial cell that produces myelin proteins and adheres to the axons providing energy and metabolic support which susceptible to endoplasmic reticulum protein quality control. Disruption of cellular protein homeostasis led to OLs dysfunction and cell death, immediately, the unfolded protein response (UPR) activated to attempt to restore the protein homeostasis via IRE1/XBP1s, PERK/eIF2α and ATF6 pathway that reduced protein translation, strengthen protein-folding capacity, and degraded unfolding/misfolded protein. Moreover, recent works have revealed the conspicuousness function of ER signaling pathways in regulating influenced factors such as calcium homeostasis, mitochondrial reactive oxygen generation, and autophagy activation to regulate protein hemostasis and improve the myelination function of OLs. Each of the regulation modes and their corresponding molecular mechanisms provides unique opportunities and distinct perspectives to obtain a deep understanding of different actions of ER stress in maintaining OLs' health and function. Therefore, our review focuses on summarizing the current understanding of ER stress on OLs' protein homeostasis micro-environment in myelination during white matter development, as well as the pathophysiology of WMI, and discussing the further potential experimental therapeutics targeting these factors that restore the function of the UPR in OLs myelination function.
早产儿脑白质损伤(WMI)是一种脱髓鞘疾病,早产儿发病率和死亡率高。少突胶质细胞(OLs)是一种专门的神经胶质细胞,可产生髓鞘蛋白并附着在轴突上,提供能量和代谢支持,易受内质网蛋白质量控制的影响。细胞内蛋白质平衡的破坏导致 OLs 功能障碍和细胞死亡,随后未折叠蛋白反应(UPR)被激活,通过 IRE1/XBP1s、PERK/eIF2α 和 ATF6 途径尝试恢复蛋白质平衡,减少蛋白质翻译、增强蛋白质折叠能力和降解未折叠/错误折叠的蛋白质。此外,最近的研究工作揭示了 ER 信号通路在调节钙稳态、线粒体活性氧生成和自噬激活等影响因素方面的显著功能,以调节蛋白质平衡并改善 OLs 的髓鞘形成功能。每种调节模式及其相应的分子机制为深入了解 ER 应激在维持 OLs 健康和功能方面的不同作用提供了独特的机会和不同的视角。因此,我们的综述重点总结了内质网应激对髓鞘形成过程中 OLs 蛋白质平衡微环境的当前认识,以及 WMI 的病理生理学,并讨论了针对这些因素的进一步潜在实验治疗方法,这些因素可以恢复 UPR 在 OLs 髓鞘形成功能中的作用。