转运蛋白颗粒复合体在囊泡拴系中的作用与相关疾病(英文)  

A perspective from transport protein particle: Vesicle tether and human diseases

作　　者：李春满[1] 余小彬[1,2] 

机构地区：[1]香港中文大学生物医学学院 [2]香港中文大学生物医学学院上皮细胞研究中心

出　　处：《生理学报》2014年第1期1-6,共6页Acta Physiologica Sinica

摘　　要：在真核细胞中,囊泡介导的蛋白质转运是一个高度可控的多步骤过程。在囊泡与靶细胞器膜成分融合之前,许多因子参与了它们之间的特异性识别和拴系。其中大部分由多亚基复合体或卷曲螺旋蛋白构成的拴系因子,在小G蛋白的协助下,介导了囊泡与靶细胞器膜成分之间最初的结合。转运蛋白颗粒(transport protein particle,TRAPP)复合体就是一种广泛参与囊泡在细胞内转运的多亚基拴系因子。本文将就TRAPP复合体结构与功能的最新研究进展及与TRAPP复合体基因突变相关疾病做一简单综述和总结。Vesicle-mediated transport of proteins is a highly regulated, multi-step process. When the vesicle is approaching its target membrane compartment, many factors are required to provide specificity and tethering between the incoming vesicle and the target membrane, before vesicle fusion can occur. Tethering factors, which include multisubunit complexes, coiled-coil proteins, with the help of small GTPases, provide the initial interaction between the vesicle and its target membrane. Of the multisubunit tethering fac- tors, the transport protein particle （TRAPP） complexes function in a number of trafficking steps, including endoplasmic reticulum （ER）-to-Golgi transport, intra- and post-Golgi traffic and autophagosome formation. In this review, we summarize the updated progress in structure and function of TRAPP complexes as well as human diseases caused by genetic mutations in TRAPP.

关 键 词：转运蛋白颗粒复合体 囊泡 COPII衣被蛋白 精神发育迟滞 肢带型肌营养不良症 

分 类 号：R346[医药卫生—基础医学]