Medicines Discovery Institute, Cardiff University, Cardiff CF10 3AT, United Kingdom.
Medicines Discovery Institute, Cardiff University, Cardiff CF10 3AT, United Kingdom.
Biochim Biophys Acta Mol Cell Res. 2021 Jul;1868(8):119058. doi: 10.1016/j.bbamcr.2021.119058. Epub 2021 May 12.
All cells contain ribonucleoprotein (RNP) granules - large membraneless structures composed of RNA and proteins. Recent breakthroughs in RNP granule research have brought a new appreciation of their crucial role in organising virtually all cellular processes. Cells widely exploit the flexible, dynamic nature of RNP granules to adapt to a variety of functional states and the ever-changing environment. Constant exchange of molecules between the different RNP granules connects them into a network. This network controls basal cellular activities and is remodelled to enable efficient stress response. Alterations in RNP granule structure and regulation have been found to lead to fatal human diseases. The interconnectedness of RNP granules suggests that the RNP granule network as a whole becomes affected in disease states such as a representative neurodegenerative disease amyotrophic lateral sclerosis (ALS). In this review, we summarize available evidence on the communication between different RNP granules and on the RNP granule network disruption as a primary ALS pathomechanism.
所有细胞都含有核仁核糖核蛋白 (RNP) 颗粒——由 RNA 和蛋白质组成的大型无膜结构。RNP 颗粒研究的最新突破使人们对其在组织几乎所有细胞过程中的关键作用有了新的认识。细胞广泛利用 RNP 颗粒的灵活、动态特性来适应各种功能状态和不断变化的环境。不同 RNP 颗粒之间的分子不断交换将它们连接成一个网络。该网络控制着基础细胞活动，并进行重塑以实现有效的应激反应。已经发现 RNP 颗粒结构和调节的改变会导致致命的人类疾病。RNP 颗粒的相互关联性表明，在疾病状态下，整个 RNP 颗粒网络都会受到影响，例如代表性的神经退行性疾病肌萎缩侧索硬化症 (ALS)。在这篇综述中，我们总结了关于不同 RNP 颗粒之间的通信以及 RNP 颗粒网络破坏作为 ALS 主要病理机制的现有证据。
