The Sichuan Provincial Key Laboratory for Human Disease Gene Study and Department of Laboratory Medicine, Center for Medical Genetics, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China.
Qinghai Provincial Key Laboratory of Tibetan Medicine Research, Northwest Institute of Plateau Biology, Chinese Academy of Sciences, Xining, Qinghai, China.
FASEB J. 2024 Mar 31;38(6):e23539. doi: 10.1096/fj.202302266R.
The endoplasmic reticulum (ER) is the largest membrane system in eukaryotic cells and is the primary site for the biosynthesis of lipids and carbohydrates, as well as for the folding, assembly, modification, and transport of secreted and integrated membrane proteins. The ER membrane complex (EMC) on the ER membrane is an ER multiprotein complex that affects the quality control of membrane proteins, which is abundant and widely preserved. Its disruption has been found to affect a wide range of processes, including protein and lipid synthesis, organelle communication, endoplasmic reticulum stress, and viral maturation, and may lead to neurodevelopmental disorders and cancer. Therefore, EMC has attracted the attention of many scholars and become a hot field. In this paper, we summarized the main contributions of the research of EMC in the past nearly 15 years, and reviewed the structure and function of EMC as well as its related diseases. We hope this review will promote further progress of research on EMC.
内质网（ER）是真核细胞中最大的膜系统，是脂质和碳水化合物生物合成的主要场所，也是分泌和整合膜蛋白的折叠、组装、修饰和运输的主要场所。内质网膜上的内质网膜复合物（EMC）是一种内质网多蛋白复合物，影响膜蛋白的质量控制，其含量丰富且广泛保存。研究发现，它的破坏会影响包括蛋白质和脂质合成、细胞器通讯、内质网应激和病毒成熟在内的广泛过程，并可能导致神经发育障碍和癌症。因此，EMC 引起了许多学者的关注，成为一个热门领域。本文总结了近 15 年来 EMC 研究的主要贡献，并回顾了 EMC 的结构和功能及其相关疾病。我们希望这篇综述将促进 EMC 研究的进一步进展。
