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Rethinking Unconventional Translation in Neurodegeneration.

ABSTRACT: 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.

SUBMITTER: Gao FB

PROVIDER: S-EPMC5728172 | biostudies-literature | 2017 Nov

REPOSITORIES: biostudies-literature

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Rethinking Unconventional Translation in Neurodegeneration.

Gao Fen-Biao FB Richter Joel D JD Cleveland Don W DW

Cell 20171101 5

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 init ...[more]

PMID: 29149615

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