2021
Regulation of nonsense-mediated mRNA decay in neural development and disease
Lee PJ, Yang S, Sun Y, Guo JU. Regulation of nonsense-mediated mRNA decay in neural development and disease. Journal Of Molecular Cell Biology 2021, 13: 269-281. PMID: 33783512, PMCID: PMC8339359, DOI: 10.1093/jmcb/mjab022.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsMeSH KeywordsAnimalsCentral Nervous SystemCodon, NonsenseGene Expression Regulation, DevelopmentalHumansMutationNeurodevelopmental DisordersNonsense Mediated mRNA DecayProtein BiosynthesisRNA, MessengerConceptsAberrant mRNAsNonsense-mediated mRNA decayMRNA decay functionCore NMD factorsMRNA surveillance mechanismGene regulation mechanismsQuality control mechanismsPremature termination codonNMD factorsPhysiological mRNAsOrganismal levelMRNA decayDevelopmental regulationGenetic evidenceMolecular basisTermination codonBiological functionsRegulation mechanismNeural developmentPhysiological functionsSurveillance mechanismNMDNeurodegenerative diseasesMRNACritical role
2020
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-SeqRNA, MessengerTrans-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