Relax, Don’t RAN Translate It

The (GGGGCC)n repeat expansion in C9orf72, which is the most common cause of frontotemporal dementia and amyotrophic lateral sclerosis, is translated through repeat-associated non-AUG (RAN) translation. In this issue of Neuron, Cheng et al. (2019) report that the helicase DDX3X, which unwinds (or re...

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Bibliographic Details
Published in:Neuron (Cambridge, Mass.) Mass.), 2019-12, Vol.104 (5), p.827-829
Main Authors: Wilson, Katherine M., Muralidharan, Bhavana, Isaacs, Adrian M.
Format: Article
Language:English
Subjects:
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - genetics
C9orf72 Protein
Cell cycle
CRISPR
CRISPR-Cas Systems
DEAD-box RNA Helicases
Dementia disorders
DNA helicase
DNA Repeat Expansion
Frontotemporal dementia
Frontotemporal Dementia - genetics
Genomes
Humans
Insects
Neurons
Ribonucleic acid
RNA
RNA Helicases
Toxicity
Translation
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Summary:The (GGGGCC)n repeat expansion in C9orf72, which is the most common cause of frontotemporal dementia and amyotrophic lateral sclerosis, is translated through repeat-associated non-AUG (RAN) translation. In this issue of Neuron, Cheng et al. (2019) report that the helicase DDX3X, which unwinds (or relaxes) RNA, suppresses RAN translation and toxicity. The (GGGGCC)n repeat expansion in C9orf72, which is the most common cause of frontotemporal dementia and amyotrophic lateral sclerosis, is translated through repeat-associated non-AUG (RAN) translation. In this issue of Neuron, Cheng et al. (2019) report that the helicase DDX3X, which unwinds (or relaxes) RNA, suppresses RAN translation and toxicity.
ISSN:0896-6273
1097-4199
DOI:10.1016/j.neuron.2019.11.014