C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression
Sun Y, Eshov A, Zhou J, Isiktas AU, Guo JU. C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression. Nature Communications 2020, 11: 3354. PMID: 32620797, PMCID: PMC7335171, DOI: 10.1038/s41467-020-17129-0.Peer-Reviewed Original ResearchMeSH KeywordsAmyotrophic Lateral SclerosisAnimalsC9orf72 ProteinCell Line, TumorCell SurvivalDatasets as TopicDNA Repeat ExpansionEmbryo, MammalianFemaleFrontal LobeFrontotemporal DementiaHumansIntronsMiceNeuronsNonsense Mediated mRNA DecayPrimary Cell CultureProtein BiosynthesisRNA HelicasesRNA, MessengerRNA-SeqTrans-ActivatorsConceptsArginine-rich dipeptide repeatsNonsense-mediated decayRNA surveillanceTranslational repressionNMD inhibitionDipeptide repeatsRNA Decay mechanismsGlobal translational repressionStress granule formationC9ALS/FTDRNA decayFrameshift 1Repeat regionFamilial amyotrophic lateral sclerosisGranule formationCultured cellsFTD brainC9orf72 geneRepressionSurvival of neuronsRepeatsAmyotrophic lateral sclerosisMutantsGenesLateral sclerosis