内质网滞留蛋白1功能的研究进展

Research advances in the function of retention in endoplasmic reticulum 1

内质网滞留蛋白1(RER1)是位于顺式高尔基体和内质网-高尔基体中间体的一个分子量为23×103的内质网滞留蛋白。作为蛋白质早期分泌途径的重要质量控制因子之一,RER1识别底物跨膜结构域的内质网滞留信号,通过外被蛋白质复合体Ⅰ将底物从高尔基体逆向转运至内质网。近20年来,RER1的底物陆续在酵母和哺乳动物细胞中被发现。这些底物通常是膜蛋白,包括永久性或暂时性内质网滞留蛋白和多聚复合物的未组装亚基。目前研究表明,RER1通过底物的内质网滞留信号,调节底物的正确定位和充分组装,影响其细胞膜表达水平或引发内质网应激,参与某些疾病的发病机制。本文总结、分析了RER1的功能、底物及其相关疾病,为RER1的未来研究方向提供参考。

Retention in endoplasmic reticulum 1(RER1)is a 23×103 endoplasmic reticulum protein that localizes to cis-Golgi apparatus and endoplasmic reticulum-Golgi apparatus intermediate compartment.As an important quality control factor of early protein secretion,RER1 recognizes endoplasmic reticulum retention signals of the transmembrane domain of substrates and then retrieves the substrates from the Golgi apparatus to the endoplasmic reticulum via coat protein complexⅠ.In the last 20 years,substrates of RER1 have been identified in yeast and mammalian cells.These substrates are always membrane proteins,including permanent or temporary endoplasmic reticulum resident proteins and unassembled subunits of polymeric complexes.Current studies have shown that RER1 regulates correct substrate location and full assembly,affects the expression level of substrates of cell membranes or induces endoplasmic reticulum stress,and contributes to the pathogenesis of certain diseases through endoplasmic reticulum retention signal of substrates.This article summarizes the function,substrates,and RER1-related diseases to provide references for future directions in the research on RER1.