Sun Hongji, Wu Mengxue, Wang Minxin, Zhang Xiaomin, Zhu Jia
School of Basic Medicine, Kunming Medical University, Kunming, Yunnan, China.
Forensic and Pathology Laboratory, Department of Pathology, Institute of Forensic Science, Jiaxing University, Jiaxing, China.
Front Neurosci. 2022 Nov 15;16:1032607. doi: 10.3389/fnins.2022.1032607. eCollection 2022.
The endoplasmic reticulum (ER) is the largest tubular reticular organelle spanning the cell. As the main site of protein synthesis, Ca homeostasis maintenance and lipid metabolism, the ER plays a variety of essential roles in eukaryotic cells, with ER molecular chaperones participate in all these processes. In recent years, it has been reported that the abnormal expression of ER chaperones often leads to a variety of neurodevelopmental disorders (NDDs), including abnormal neuronal migration, neuronal morphogenesis, and synaptic function. Neuronal development is a complex and precisely regulated process. Currently, the mechanism by which neural development is regulated at the ER level remains under investigation. Therefore, in this work, we reviewed the recent advances in the roles of ER chaperones in neural development and developmental disorders caused by the deficiency of these molecular chaperones.
内质网（ER）是贯穿细胞的最大管状网状细胞器。作为蛋白质合成、钙稳态维持和脂质代谢的主要场所，内质网在真核细胞中发挥着多种重要作用，内质网分子伴侣参与了所有这些过程。近年来，有报道称内质网伴侣蛋白的异常表达常导致多种神经发育障碍（NDDs），包括神经元迁移异常、神经元形态发生异常和突触功能异常。神经元发育是一个复杂且精确调控的过程。目前，内质网水平调控神经发育的机制仍在研究中。因此，在这项工作中，我们综述了内质网伴侣蛋白在神经发育中的作用以及这些分子伴侣蛋白缺乏所导致的发育障碍的最新研究进展。
