Repeat-associated non-AUG (RAN) translation: insights from pathology

More than 40 different neurological diseases are caused by microsatellite repeat expansions. Since the discovery of repeat-associated non-AUG (RAN) translation by Zu et al. in 2011, nine expansion disorders have been identified as RAN-positive diseases. RAN proteins are translated from different typ...

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Bibliographic Details
Published in:Laboratory investigation 2019-07, Vol.99 (7), p.929-942
Main Authors: Banez-Coronel, Monica, Ranum, Laura P.W.
Format: Article
Language:English
Subjects:
631/378/2584
692/617/375
Animals
Antisense RNA
Brain
Gene Expression
Humans
Laboratory Medicine
Medicine
Medicine & Public Health
Microsatellite Repeats
Nervous System Diseases - genetics
Neurological diseases
Nucleotides
Pathology
Proteins
Proteostasis Deficiencies - etiology
Proteostasis Deficiencies - therapy
Ran protein
Review Article
Translation
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Description
Summary:More than 40 different neurological diseases are caused by microsatellite repeat expansions. Since the discovery of repeat-associated non-AUG (RAN) translation by Zu et al. in 2011, nine expansion disorders have been identified as RAN-positive diseases. RAN proteins are translated from different types of nucleotide repeat expansions and can be produced from both sense and antisense transcripts. In some diseases, RAN proteins have been shown to accumulate in affected brain regions. Here we review the pathological and molecular aspects associated with RAN protein accumulation for each particular disorder, the correlation between disease pathology and the available in vivo models and the common aspects shared by some of the newly discovered RAN proteins.
ISSN:0023-6837
1530-0307
DOI:10.1038/s41374-019-0241-x