Rethinking Unconventional Translation in Neurodegeneration.

Rethinking Unconventional Translation in Neurodegeneration.
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DOI:
10.1016/j.cell.2017.10.042
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发表时间:
2017-11-16
期刊:
影响因子:
64.5
通讯作者:
Cleveland DW
Cleveland DW
中科院分区:
生物学1区
文献类型:
--
作者:
Gao FB;Richter JD;Cleveland DW

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真核生物的翻译受到严格的监管,以确保蛋白质生产在正确的时间和地点发生。最近对异常重复蛋白的研究,特别是在由核苷酸重复序列扩张引起的年龄依赖性神经退行性疾病中,强调或识别了两种非常规翻译起始形式:利用AUG样位点(近同源)或重复相关的非AUG(RAN)翻译。我们讨论重复蛋白可能如何不同,不仅由于非常规的起始,而且由于核糖体移帧和/或不完善的重复DNA复制、扩张和修复,并强调对重复序列翻译的研究如何揭示翻译的生物学及其对疾病的贡献。
Eukaryotic translation is tightly regulated to ensure that protein production occurs at the right time and place. Recent studies on abnormal repeat proteins, especially in age-dependent neurodegenerative diseases caused by nucleotide repeat expansion, have highlighted or identified two forms of unconventional translation initiation: usage of AUG-like sites (near cognates) or repeat-associated non-AUG (RAN) translation. We discuss how repeat proteins may differ due to not just unconventional initiation, but also ribosomal frameshifting and/or imperfect repeat DNA replication, expansion and repair, and highlight how research on translation of repeats may uncover insights into the biology of translation and its contribution to disease.
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