Department of Cell Biology, Emory University School of Medicine, Atlanta, Georgia, USA.
Traffic. 2020 Jul;21(7):454-462. doi: 10.1111/tra.12733.
RNA granule formation, which can be regulated by RNA-binding proteins (RBPs) such as fragile X mental retardation protein (FMRP), acts as a mechanism to control both the repression and subcellular localization of translation. Dysregulated assembly of RNA granules has been implicated in multiple neurological disorders, such as amyotrophic lateral sclerosis. Thus, it is crucial to understand the cellular pathways impinging upon granule assembly or disassembly. The goal of this review is to summarize recent advances in our understanding of the role of the RBP, FMRP, in translational repression underlying RNA granule dynamics, mRNA transport and localized. We summarize the known mechanisms of translational regulation by FMRP, the role of FMRP in RNA transport granules, fragile X granules and stress granules. Focusing on the emerging link between FMRP and stress granules, we propose a model for how hyperassembly and hypoassembly of RNA granules may contribute to neurological diseases.
RNA 颗粒的形成可以被 RNA 结合蛋白（RBPs）如脆性 X 智力迟钝蛋白（FMRP）调节，作为控制翻译的抑制和亚细胞定位的机制。RNA 颗粒组装失调与多种神经疾病有关，如肌萎缩侧索硬化症。因此，了解影响颗粒组装或解组装的细胞途径至关重要。这篇综述的目的是总结我们对 RBP、FMRP 在 RNA 颗粒动力学、mRNA 运输和局部翻译抑制中的作用的理解的最新进展。我们总结了 FMRP 对翻译的已知调节机制、FMRP 在 RNA 运输颗粒、脆性 X 颗粒和应激颗粒中的作用。我们专注于 FMRP 和应激颗粒之间新出现的联系，提出了一个模型，说明 RNA 颗粒的过度组装和组装不足如何导致神经疾病。
