Zeng Hui, Liu Ye, Liu Xinjie, Li Jianwei, Lu Lixuan, Xue Cheng, Wu Xiao, Zhang Xinran, Zheng Zijian, Lu Guohui
Department of Neurosurgery, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, 330006, China.
The First Clinical Medical College, Jiangxi Medical College, Nanchang University, Nanchang, 330006, China.
Inflammation. 2024 Oct 9. doi: 10.1007/s10753-024-02156-6.
Oligomeric forms of α-synuclein (α-syn) are critical in the formation of α-synuclein fibrils, exhibiting neurotoxic properties that are pivotal in the pathogenesis of Parkinson's disease (PD). A salient feature of this pathology is the disruption of the protein folding capacity of the endoplasmic reticulum (ER), leading to a perturbation in the ER's protein quality control mechanisms. The accumulation of unfolded or misfolded proteins instigates ER stress. However, the onset of ER stress and the consequent activation of the Unfolded Protein Response (UPR) and Endoplasmic Reticulum-Associated Degradation (ERAD) pathways do not merely culminate in apoptosis when they fail to restore cellular homeostasis. More critically, this condition initiates a cascade of reactions involving ER-related structures and organelles, resulting in multifaceted cellular damage and, potentially, a feedback loop that precipitates neuroinflammation. In this review, we elucidate the interplay between UPR and ERAD, as well as the intricate crosstalk among the ER and other organelles such as mitochondria, lysosomes, and the Golgi apparatus, underscoring their roles in the neurodegenerative process.
α-突触核蛋白(α-syn)的寡聚体形式在α-突触核蛋白纤维的形成中起关键作用,表现出神经毒性特性,这在帕金森病(PD)的发病机制中至关重要。这种病理的一个显著特征是内质网(ER)蛋白质折叠能力的破坏,导致ER蛋白质质量控制机制受到干扰。未折叠或错误折叠蛋白质的积累引发内质网应激。然而,内质网应激的发生以及随之而来的未折叠蛋白反应(UPR)和内质网相关降解(ERAD)途径的激活,当它们未能恢复细胞内稳态时,不仅仅会导致细胞凋亡。更关键的是,这种情况会引发一系列涉及内质网相关结构和细胞器的反应,导致多方面的细胞损伤,并可能形成一个引发神经炎症的反馈回路。在这篇综述中,我们阐明了UPR和ERAD之间的相互作用,以及内质网与其他细胞器(如线粒体、溶酶体和高尔基体)之间复杂的串扰,强调了它们在神经退行性过程中的作用。