Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Department of Cell and Molecular Biology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Cells. 2020 Feb 11;9(2):408. doi: 10.3390/cells9020408.
Membrane and secretory proteins are essential for almost every aspect of cellular function. These proteins are incorporated into ER-derived carriers and transported to the Golgi before being sorted for delivery to their final destination. Although ER-to-Golgi trafficking is highly conserved among eukaryotes, several layers of complexity have been added to meet the increased demands of complex cell types in metazoans. The specialized morphology of neurons and the necessity for precise spatiotemporal control over membrane and secretory protein localization and function make them particularly vulnerable to defects in trafficking. This review summarizes the general mechanisms involved in ER-to-Golgi trafficking and highlights mutations in genes affecting this process, which are associated with neurological diseases in humans.
膜蛋白和分泌蛋白对于细胞功能的几乎各个方面都是必不可少的。这些蛋白被整合到内质网衍生的载体中，并在被分拣以运送到最终目的地之前被运送到高尔基体。尽管内质网到高尔基体的运输在真核生物中高度保守，但已经增加了几个层次的复杂性，以满足后生动物中复杂细胞类型的增加需求。神经元的特殊形态以及对膜和分泌蛋白定位和功能的精确时空控制的必要性，使它们特别容易受到运输缺陷的影响。这篇综述总结了涉及内质网到高尔基体运输的一般机制，并强调了影响该过程的基因突变，这些基因突变与人类的神经疾病有关。
