Chen Guofang, Wei Tingyi, Ju Furong, Li Haisen
Shanghai Key Laboratory of Maternal Fetal Medicine, Clinical and Translational Research Center of Shanghai First Maternity and Infant Hospital, Tongji University School of Medicine, Shanghai, China.
Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Front Cell Dev Biol. 2023 Apr 19;11:1156152. doi: 10.3389/fcell.2023.1156152. eCollection 2023.
Endoplasmic reticulum (ER) is the largest membrane-bound compartment in all cells and functions as a key regulator in protein biosynthesis, lipid metabolism, and calcium balance. Mammalian endoplasmic reticulum has evolved with an orchestrated protein quality control system to handle defective proteins and ensure endoplasmic reticulum homeostasis. Nevertheless, the accumulation and aggregation of misfolded proteins in the endoplasmic reticulum may occur during pathological conditions. The inability of endoplasmic reticulum quality control system to clear faulty proteins and aggregates from the endoplasmic reticulum results in the development of many human disorders. The efforts to comprehensively understand endoplasmic reticulum quality control network and protein aggregation will benefit the diagnostics and therapeutics of endoplasmic reticulum storage diseases. Herein, we overview recent advances in mammalian endoplasmic reticulum protein quality control system, describe protein phase transition model, and summarize the approaches to monitor protein aggregation. Moreover, we discuss the therapeutic applications of enhancing endoplasmic reticulum protein quality control pathways in endoplasmic reticulum storage diseases.
内质网（ER）是所有细胞中最大的膜结合区室，在蛋白质生物合成、脂质代谢和钙平衡中起着关键调节作用。哺乳动物内质网已经进化出一个精心编排的蛋白质质量控制系统，以处理有缺陷的蛋白质并确保内质网的稳态。然而，在病理条件下，内质网中错误折叠蛋白质的积累和聚集可能会发生。内质网质量控制系统无法从内质网中清除错误的蛋白质和聚集体会导致许多人类疾病的发生。全面了解内质网质量控制网络和蛋白质聚集的努力将有利于内质网储存疾病的诊断和治疗。在此，我们概述了哺乳动物内质网蛋白质质量控制系统的最新进展，描述了蛋白质相变模型，并总结了监测蛋白质聚集的方法。此外，我们还讨论了增强内质网蛋白质质量控制途径在内质网储存疾病中的治疗应用。
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