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中华妇幼临床医学杂志(电子版) ›› 2020, Vol. 16 ›› Issue (04) : 373 -380. doi: 10.3877/cma.j.issn.1673-5250.2020.04.001

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内质网应激及Wolframin蛋白质与妊娠相关疾病研究现状
徐婷婷1, 王晓东1,†()   
  1. 1. 四川大学华西第二医院儿科、出生缺陷与相关妇儿疾病教育部重点实验室，成都　610041
  • 收稿日期:2020-01-03 修回日期:2020-06-30 出版日期:2020-08-01
  • 通信作者: 王晓东

Current research progresses of endoplasmic reticulum stress, Wolframin protein and pregnancy-related diseases

Tingting Xu1, Xiaodong Wang1,†()   

  1. 1. Department of Obstetrics and Gynecology, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
  • Received:2020-01-03 Revised:2020-06-30 Published:2020-08-01
  • Corresponding author: Xiaodong Wang
  • About author:
    Corresponding author: Wang Xiaodong, Email:
  • Supported by:
    National Natural Science Foundation of China(81571446)
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徐婷婷, 王晓东. 内质网应激及Wolframin蛋白质与妊娠相关疾病研究现状[J]. 中华妇幼临床医学杂志(电子版), 2020, 16(04): 373-380.

Tingting Xu, Xiaodong Wang. Current research progresses of endoplasmic reticulum stress, Wolframin protein and pregnancy-related diseases[J]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2020, 16(04): 373-380.

内质网应激(ERS)是细胞应激反应的核心环节。根据ERS持续时间，可将其分为急性ERS和慢性ERS 2种类型。根据诱发ERS的原因，可将其分为未折叠蛋白质反应(UPR)、内质网过度负荷反应(EOR)和固醇调节元件结合蛋白质(SREBP)通路调节反应3种类型。WFS1基因编码的跨膜糖蛋白质Wolframin蛋白质，是内质网上的常驻跨膜蛋白质，亦是参与调控ERS的蛋白质。目前，对ERS的研究主要集中在缺血缺氧、葡萄糖缺乏、脂质过度负荷、Ca2＋紊乱等方面；对Wolframin蛋白质的相关研究，则主要集中在Wolfram综合征相应典型疾病，如糖尿病、视神经萎缩、双相情感障碍等方面。围生医学领域ERS与妊娠相关并发症密切相关，但是由于方法学等的滞涸，目前对ERS与子痫前期、妊娠期糖尿病(GDM)、妊娠期肝内胆汁淤积症等妊娠相关疾病研究的文献报道较少。笔者拟就激活ERS分类、Wolframin蛋白质与ERS关系，ERS介导的相应信号通路，Wolframin蛋白质在上述信号通路中的相应调控作用的最新研究等进行阐述，旨在为妊娠相关疾病的病理生理机制研究提供新的方向。

关键词: 内质网, 内质网应激, Wolfram综合征, Wolframin蛋白质

Endoplasmic reticulum stress (ERS) is the core of cellular stress response. It can be classified into acute ERS and chronic ERS depending on the duration of ERS. Besides, it also can be divided into unfolded protein reaction (UPR), endoplasmic reticulum overload response (EOR), and sterol-regulatory element binding protein (SREBP) according to the different inducements of ERS. Wolframin protein, an resident membrane glycoprotein of endoplasmic reticulum, plays a key role in regulating ERS to maintain endoplasmic reticulum homeostasis by affecting both gene transcription and protein translation. At present, researches on ERS are mainly focused on ischemia, hypoxia, glucose deficiency, lipid overload, calcium ion disorder, etc., while researches on Wolframin proteins are mainly focused on the typical diseases of Wolfram syndrome including diabetes mellitus, optic atrophy, and bipolar disorder. In the field of perinatal medicine, ERS is closely related to pregnancy-related complications. However, due to stagnation of methodology, there are few researches about ERS and pregnancy-related diseases, such as preeclampsia, gestational diabetes mellitus (GDM), intrahepatic cholestasis of pregnancy. Hence, we report the latest related research of the classification of ERS, the relationship between Wolframin proteins and ERS, the corresponding signaling pathways mediated by ERS, and corresponding regulatory role of Wolframin proteins in the above signaling pathways, in order to provide a new direction for the pathophysiological mechanism of pregnance-related diseases.

Key words: Endoplasmic reticulum, Endoplasmic reticulum stress, Wolfram syndrome, Wolframin protein
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