RNA Granules and Diseases: A Case Study of Stress Granules in ALS and FTLD.
RNA Granules and Diseases: A Case Study of Stress Granules in ALS and FTLD.
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RNA颗粒和疾病：ALS和FTLD中应力颗粒的案例研究。
DOI：
10.1007/978-3-319-29073-7_11
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发表时间：
2016
影响因子：
--
通讯作者：
Leung AK
中科院分区：
文献类型：
--
作者：
Fan AC;Leung AK
RNA颗粒是显微镜下可见的细胞结构，通过蛋白质-蛋白质和蛋白质-RNA相互作用聚集。以应激颗粒为例，我们讨论了RNA颗粒形成的原理，它依赖于RNA和多结构域蛋白的多价性以及与朊病毒样/低复杂性结构域（例如FUS和TDP-43）的蛋白质之间的低亲和力相互作用。然后，我们探讨RNA颗粒形成的失调如何与神经退行性疾病，如肌萎缩侧索硬化症（ALS）和额颞叶变性（FTLD），并讨论可能的治疗干预策略。
RNA granules are microscopically visible cellular structures that aggregate by protein–protein and protein-RNA interactions. Using stress granules as an example, we discuss the principles of RNA granule formation, which rely on the multivalency of RNA and multi-domain proteins as well as low-affinity interactions between proteins with prion-like/low-complexity domains (e.g. FUS and TDP-43). We then explore how dysregulation of RNA granule formation is linked to neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), and discuss possible strategies for therapeutic intervention.