Journal
CURRENT OPINION IN GENETICS & DEVELOPMENT
Volume 44, Issue -, Pages 125-134Publisher
CURRENT BIOLOGY LTD
DOI: 10.1016/j.gde.2017.03.006
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Funding
- National Institutes of Health [RO1 NS098819, R01 NS040389, P01 NS058901]
- Target ALS
- CHDI
- National Ataxia Foundation
- ALS Association
- Packard Foundation
- The Marigold Foundation
- Muscular Dystrophy Association
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Since the discovery of repeat-associated non-ATG (RAN) translation, and more recently its association with amyotrophic lateral sclerosis/frontotemporal dementia, there has been an intense focus to understand how this process works and the downstream effects of these novel proteins. RAN translation across several different types of repeat expansions mutations (CAG, CTG, CCG, GGGGCC, GGCCCC) results in the production of proteins in all three reading frames without an ATG initiation codon. The combination of bidirectional transcription and RAN translation has been shown to result in the accumulation of up to six mutant expansion proteins in a growing number of diseases. This process is complex mechanistically and also complex from the perspective of the downstream consequences in disease. Here we review recent developments in RAN translation and their implications on our basic understanding of neurodegenerative disease and gene expression.
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