Telethon Institute of Genetics and Medicine, Via Pietro Castellino 111, Naples 80131, Italy.
Telethon Institute of Genetics and Medicine, Via Pietro Castellino 111, Naples 80131, Italy.
Trends Cell Biol. 2014 Jan;24(1):9-18. doi: 10.1016/j.tcb.2013.08.005. Epub 2013 Sep 26.
The vast majority of proteins that are transported to different cellular compartments and secreted from the cell require coat protein complex II (COPII) for export from the endoplasmic reticulum (ER). Many of the molecular mechanisms underlying COPII assembly are understood in great detail, but it is becoming increasingly evident that this basic machinery is insufficient to account for diverse aspects of protein export from the ER that are observed in vivo. Here we review recent data that have furthered our mechanistic understanding of COPII assembly and, in particular, how genetic diseases associated with the early secretory pathway have added fundamental insights into the regulation of ER-derived carrier formation. We also highlight some unresolved issues that future work should address to better understand the physiology of COPII-mediated transport.
绝大多数被运输到不同细胞区室并从细胞中分泌的蛋白质都需要衣壳蛋白复合物 II (COPII) 从内质网 (ER) 中输出。许多 COPII 组装的分子机制都被详细地理解了，但是越来越明显的是，这个基本的机械装置不足以解释在体内观察到的 ER 中蛋白质输出的不同方面。在这里，我们回顾了最近的数据，这些数据进一步加深了我们对 COPII 组装的机制理解，特别是与早期分泌途径相关的遗传疾病如何为 ER 衍生载体形成的调节提供了基本的见解。我们还强调了一些未来工作应该解决的未解决问题，以更好地理解 COPII 介导的运输的生理学。
