Measuring Repeat-Associated Non-AUG (RAN) Translation.
Measuring Repeat-Associated Non-AUG (RAN) Translation.
复制标题
测量重复相关的非 AUG (RAN) 翻译。
DOI：
10.1007/978-1-0716-1975-9_8
复制
发表时间：
2022
期刊：
影响因子：
--
通讯作者：
Sun,Shuying
中科院分区：
文献类型：
--
作者：
Wang,Shaopeng;Sun,Shuying
短核苷酸重复序列的扩增导致了50多种神经或神经肌肉疾病。许多含有重复扩增的RNA可以通过所有阅读框中的重复相关非AUG（RAN）翻译产生毒性重复蛋白。了解RAN翻译如何发生以及哪些细胞因子调节这一过程将有助于破译分子过程和疾病发病机制的基本机制。使用报告系统定量测量RAN翻译提供了一个平台，以检查候选基因/途径和筛选这种非经典途径的修饰剂。在本章中，我们描述了双荧光素酶报告系统来测量RAN翻译使用C9 ORF 72 GGGGCCexpas为例，这是最常见的肌萎缩侧索硬化症（ALS）和额颞叶痴呆症（FTD）的遗传原因。
Expansions of short nucleotide repeats account for more than 50 neurological or neuromuscular diseases. Many repeat expansion-containing RNAs can generate toxic repeat proteins through repeat-associated non-AUG (RAN) translation in all the reading frames. Understanding how RAN translation occurs and what cellular factors regulate this process will help decipher the basic mechanism of the molecular process and disease pathogenesis. Using reporter systems to quantitatively measure RAN translation provides a platform to examine candidate genes/pathways and screen for modifiers of this non-canonical pathway. In this chapter, we describe the dual-luciferase reporter system to measure RAN translation usingC9ORF72GGGGCCexpas an example, which is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).