Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, Colorado 80303; email:
Annu Rev Biochem. 2015;84:791-811. doi: 10.1146/annurev-biochem-072711-163501. Epub 2015 Jan 12.
The endoplasmic reticulum (ER) has a remarkably complex structure, composed of a single bilayer that forms the nuclear envelope, along with a network of sheets and dynamic tubules. Our understanding of the biological significance of the complex architecture of the ER has improved dramatically in the last few years. The identification of proteins and forces required for maintaining ER shape, as well as more advanced imaging techniques, has allowed the relationship between ER shape and function to come into focus. These studies have also revealed unexpected new functions of the ER and novel ER domains regulating alterations in ER dynamics. The importance of ER structure has become evident as recent research has identified diseases linked to mutations in ER-shaping proteins. In this review, we discuss what is known about the maintenance of ER architecture, the relationship between ER structure and function, and diseases associated with defects in ER structure.
内质网(ER)具有非常复杂的结构,由单层双层组成核膜,以及片状和动态管状网络。在过去的几年中,我们对内质网复杂结构的生物学意义的理解有了显著的提高。鉴定维持内质网形状所需的蛋白质和力,以及更先进的成像技术,使内质网形状和功能之间的关系成为焦点。这些研究还揭示了内质网的意外新功能和调节内质网动力学变化的新型内质网域。内质网结构的重要性已经很明显,因为最近的研究已经确定了与内质网成形蛋白突变相关的疾病。在这篇综述中,我们讨论了内质网结构维持、内质网结构与功能之间的关系以及与内质网结构缺陷相关的疾病方面的已知内容。