Rethinking Unconventional Translation in Neurodegeneration

被引：55

作者：

Gao, Fen-Biao ^{[1
]}

Richter, Joel D. ^{[2
]}

Cleveland, Don W. ^{[3
,4
,5
]}

机构：

[1] Univ Massachusetts, Sch Med, Dept Neurol, Worcester, MA 01605 USA

[2] Univ Massachusetts, Sch Med, Program Mol Med, Worcester, MA 01605 USA

[3] Univ Calif San Diego, Ludwig Inst Canc Res, La Jolla, CA 92093 USA

[4] Univ Calif San Diego, Dept Neurosci, La Jolla, CA 92093 USA

[5] Univ Calif San Diego, Dept Cellular & Mol Med, La Jolla, CA 92093 USA

来源：

CELL | 2017年 / 171卷 / 05期

关键词：

OPEN READING FRAMES; DIPEPTIDE-REPEAT PROTEINS; MESSENGER-RNA TRANSLATION; G-QUADRUPLEX STRUCTURES; TREMOR ATAXIA SYNDROME; RAN TRANSLATION; FRONTOTEMPORAL DEMENTIA; NUCLEOTIDE RESOLUTION; HEXANUCLEOTIDE REPEAT; DNA-DAMAGE;

D O I：

10.1016/j.cell.2017.10.042

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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 we highlight how research on translation of repeats may uncover insights into the biology of translation and its contribution to disease.

引用

页码：994 / 1000

页数：7

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