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Repeat-Associated Non-ATG Translation: Molecular Mechanisms and Contribution to Neurological Disease

Authors
  • Nguyen, Lien
  • Cleary, John Douglas
  • Ranum, Laura P.W.
Type
Published Article
Journal
Annual Review of Neuroscience
Publisher
Annual Reviews
Publication Date
Jul 08, 2019
Volume
42
Pages
227–247
Identifiers
DOI: 10.1146/annurev-neuro-070918-050405
Source
Annual Reviews
Keywords
License
Yellow

Abstract

Microsatellite mutations involving the expansion of tri-, tetra-, penta-, or hexanucleotide repeats cause more than 40 different neurological disorders. Although, traditionally, the position of the repeat within or outside of an open reading frame has been used to focus research on disease mechanisms involving protein loss of function, protein gain of function, or RNA gain of function, the discoveries of bidirectional transcription and repeat-associated non-ATG (RAN) have blurred these distinctions. Here we review what is known about RAN proteins in disease, the mechanisms by which they are produced, and the novel therapeutic opportunities they provide.

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