{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T18:18:19Z","timestamp":1790187499589,"version":"4.1.0"},"reference-count":121,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2020,4,1]],"date-time":"2020-04-01T00:00:00Z","timestamp":1585699200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,4,1]],"date-time":"2020-04-01T00:00:00Z","timestamp":1585699200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,4,8]],"date-time":"2021-04-08T00:00:00Z","timestamp":1617840000000},"content-version":"vor","delay-in-days":372,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/501100004923","name":"AFM-T\u00e9l\u00e9thon","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004923","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003194","name":"New York Stem Cell Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100003194","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007030","name":"Les Turner ALS Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007030","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neuron"],"published-print":{"date-parts":[[2020,4]]},"DOI":"10.1016\/j.neuron.2020.01.020","type":"journal-article","created":{"date-parts":[[2020,2,13]],"date-time":"2020-02-13T10:32:25Z","timestamp":1581589945000},"page":"90-107.e13","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":85,"title":["Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS\/FTD C9orf72 Toxicity"],"prefix":"10.1016","volume":"106","author":[{"given":"Juan A.","family":"Ortega","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth L.","family":"Daley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sukhleen","family":"Kour","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisa","family":"Samani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liana","family":"Tellez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haley S.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Hall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y. Taylan","family":"Esengul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yung-Hsu","family":"Tsai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tania F.","family":"Gendron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher J.","family":"Donnelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teepu","family":"Siddique","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey N.","family":"Savas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Udai B.","family":"Pandey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evangelos","family":"Kiskinis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.neuron.2020.01.020_bib1","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1186\/1477-5956-9-30","article-title":"A guide through the computational analysis of isotope-labeled mass spectrometry-based quantitative proteomics data: an application study","volume":"9","author":"Albaum","year":"2011","journal-title":"Proteome Sci."},{"key":"10.1016\/j.neuron.2020.01.020_bib2","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1038\/ng.2434","article-title":"Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models","volume":"44","author":"Armakola","year":"2012","journal-title":"Nat. Genet."},{"key":"10.1016\/j.neuron.2020.01.020_bib3","doi-asserted-by":"crossref","first-page":"e56","DOI":"10.1002\/cpcb.56","article-title":"Expansion microscopy: protocols for imaging proteins and RNA in cells and tissues","volume":"80","author":"Asano","year":"2018","journal-title":"Curr. Protoc. Cell Biol."},{"key":"10.1016\/j.neuron.2020.01.020_bib4","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1038\/s41582-018-0047-2","article-title":"C9orf72-mediated ALS and FTD: multiple pathways to disease","volume":"14","author":"Balendra","year":"2018","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.neuron.2020.01.020_bib5","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1038\/nchembio.1563","article-title":"Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models","volume":"10","author":"Barmada","year":"2014","journal-title":"Nat. Chem. Biol."},{"key":"10.1016\/j.neuron.2020.01.020_bib6","doi-asserted-by":"crossref","first-page":"7821","DOI":"10.1073\/pnas.1509744112","article-title":"Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1","volume":"112","author":"Barmada","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.01.020_bib7","first-page":"8219","article-title":"Mammalian nonsense codons can be cis effectors of nuclear mRNA half-life","volume":"14","author":"Belgrader","year":"1994","journal-title":"Mol. Cell. Biol."},{"issue":"D1","key":"10.1016\/j.neuron.2020.01.020_bib8","doi-asserted-by":"crossref","first-page":"D503","DOI":"10.1093\/nar\/gkx1021","article-title":"NLSdb-major update for database of nuclear localization signals and nuclear export signals","volume":"46","author":"Bernhofer","year":"2018","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib9","doi-asserted-by":"crossref","first-page":"bau012","DOI":"10.1093\/database\/bau012","article-title":"COMPARTMENTS: unification and visualization of protein subcellular localization evidence","volume":"2014","author":"Binder","year":"2014","journal-title":"Database (Oxford)"},{"key":"10.1016\/j.neuron.2020.01.020_bib10","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1016\/j.molcel.2017.02.013","article-title":"Phase separation of C9orf72 dipeptide repeats perturbs stress granule dynamics","volume":"65","author":"Boeynaems","year":"2017","journal-title":"Mol. Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib11","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1038\/nbt.1783","article-title":"A functionally characterized test set of human induced pluripotent stem cells","volume":"29","author":"Boulting","year":"2011","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.neuron.2020.01.020_bib12","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1038\/nature14896","article-title":"Structural basis for stop codon recognition in eukaryotes","volume":"524","author":"Brown","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib13","doi-asserted-by":"crossref","first-page":"5583","DOI":"10.1038\/s41467-019-13383-z","article-title":"Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization","volume":"10","author":"Casci","year":"2019","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.01.020_bib14","doi-asserted-by":"crossref","first-page":"8694","DOI":"10.1021\/ac800606w","article-title":"Comparisons of mass spectrometry compatible surfactants for global analysis of the mammalian brain proteome","volume":"80","author":"Chen","year":"2008","journal-title":"Anal. Chem."},{"key":"10.1016\/j.neuron.2020.01.020_bib15","first-page":"Unit 13.14","article-title":"Validation of tandem mass spectrometry database search results using DTASelect","volume":"Chapter 13","author":"Cociorva","year":"2007","journal-title":"Curr. Protoc. Bioinformatics"},{"key":"10.1016\/j.neuron.2020.01.020_bib16","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1261\/rna.059055.116","article-title":"Transcriptome-wide identification of NMD-targeted human mRNAs reveals extensive redundancy between SMG6- and SMG7-mediated degradation pathways","volume":"23","author":"Colombo","year":"2017","journal-title":"RNA"},{"key":"10.1016\/j.neuron.2020.01.020_bib17","doi-asserted-by":"crossref","first-page":"2040","DOI":"10.1093\/brain\/awu120","article-title":"Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions","volume":"137","author":"Cooper-Knock","year":"2014","journal-title":"Brain"},{"key":"10.1016\/j.neuron.2020.01.020_bib18","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1007\/s00401-015-1429-9","article-title":"Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy","volume":"130","author":"Cooper-Knock","year":"2015","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.neuron.2020.01.020_bib19","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.cell.2008.11.037","article-title":"Age-dependent deterioration of nuclear pore complexes causes a loss of nuclear integrity in postmitotic cells","volume":"136","author":"D\u2019Angelo","year":"2009","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib20","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.neuron.2011.09.011","article-title":"Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS","volume":"72","author":"DeJesus-Hernandez","year":"2011","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib21","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/nature10353","article-title":"Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS\/dementia","volume":"477","author":"Deng","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib22","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/j.neuron.2013.10.015","article-title":"RNA toxicity from the ALS\/FTD C9ORF72 expansion is mitigated by antisense intervention","volume":"80","author":"Donnelly","year":"2013","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib23","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1016\/j.neuron.2018.07.039","article-title":"Tau protein disrupts nucleocytoplasmic transport in Alzheimer\u2019s disease","volume":"99","author":"Eftekharzadeh","year":"2018","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib24","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1016\/1044-0305(94)80016-2","article-title":"An approach to correlate tandem mass spectral data of peptides with amino acid sequences in a protein database","volume":"5","author":"Eng","year":"1994","journal-title":"J.\u00a0Am. Soc. Mass Spectrom."},{"key":"10.1016\/j.neuron.2020.01.020_bib25","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1038\/nature14974","article-title":"GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport","volume":"525","author":"Freibaum","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib26","doi-asserted-by":"crossref","first-page":"e0176219","DOI":"10.1371\/journal.pone.0176219","article-title":"A selected reaction monitoring mass spectrometric assessment of biomarker candidates diagnosing large-cell neuroendocrine lung carcinoma by the scaling method using endogenous references","volume":"12","author":"Fukuda","year":"2017","journal-title":"PLoS ONE"},{"key":"10.1016\/j.neuron.2020.01.020_bib27","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.neuron.2017.03.027","article-title":"Polyglutamine-expanded Huntingtin exacerbates age-related disruption of nuclear integrity and nucleocytoplasmic transport","volume":"94","author":"Gasset-Rosa","year":"2017","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib28","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1007\/s00401-013-1237-z","article-title":"Mechanisms of toxicity in C9FTLD\/ALS","volume":"127","author":"Gendron","year":"2014","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.neuron.2020.01.020_bib29","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.brainres.2016.04.004","article-title":"There has been an awakening: emerging mechanisms of C9orf72 mutations in FTD\/ALS","volume":"1647","author":"Gitler","year":"2016","journal-title":"Brain Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib30","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1186\/s40478-015-0218-y","article-title":"Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration","volume":"3","author":"Gomez-Deza","year":"2015","journal-title":"Acta Neuropathol. Commun."},{"key":"10.1016\/j.neuron.2020.01.020_bib31","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1038\/s41593-019-0396-1","article-title":"Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD","volume":"22","author":"Goodman","year":"2019","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib32","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.neuron.2017.03.023","article-title":"Mutant Huntingtin disrupts the nuclear pore complex","volume":"94","author":"Grima","year":"2017","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib33","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1038\/nature13124","article-title":"C9orf72 nucleotide repeat structures initiate molecular cascades of disease","volume":"507","author":"Haeusler","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib34","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1038\/nrn.2016.38","article-title":"The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease","volume":"17","author":"Haeusler","year":"2016","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib35","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/S1097-2765(03)00105-9","article-title":"An integrated stress response regulates amino acid metabolism and resistance to oxidative stress","volume":"11","author":"Harding","year":"2003","journal-title":"Mol. Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib36","doi-asserted-by":"crossref","first-page":"e201800070","DOI":"10.26508\/lsa.201800070","article-title":"Proteomics and C9orf72 neuropathology identify ribosomes as poly-GR\/PR interactors driving toxicity","volume":"1","author":"Hartmann","year":"2018","journal-title":"Life Sci. Alliance"},{"key":"10.1016\/j.neuron.2020.01.020_bib37","doi-asserted-by":"crossref","first-page":"16063","DOI":"10.1038\/ncomms16063","article-title":"SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits","volume":"8","author":"Hautbergue","year":"2017","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.01.020_bib38","doi-asserted-by":"crossref","first-page":"11361","DOI":"10.1021\/acs.analchem.5b02721","article-title":"Extracting accurate precursor information for tandem mass spectra by RawConverter","volume":"87","author":"He","year":"2015","journal-title":"Anal. Chem."},{"key":"10.1016\/j.neuron.2020.01.020_bib39","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1002\/wrna.1137","article-title":"Regulation of nonsense-mediated mRNA decay","volume":"3","author":"Huang","year":"2012","journal-title":"Wiley Interdiscip. Rev. RNA"},{"key":"10.1016\/j.neuron.2020.01.020_bib40","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1186\/1471-2164-9-488","article-title":"BioVenn: a web application for the comparison and visualization of biological lists using area-proportional Venn diagrams","volume":"9","author":"Hulsen","year":"2008","journal-title":"BMC Genomics"},{"key":"10.1016\/j.neuron.2020.01.020_bib41","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1101\/gr.206060.116","article-title":"A GC-rich sequence feature in the 3\u2032 UTR directs UPF1-dependent mRNA decay in mammalian cells","volume":"27","author":"Imamachi","year":"2017","journal-title":"Genome Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib42","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1038\/emboj.2008.17","article-title":"Interactions between UPF1, eRFs, PABP and the exon junction complex suggest an integrated model for mammalian NMD pathways","volume":"27","author":"Ivanov","year":"2008","journal-title":"EMBO J."},{"key":"10.1016\/j.neuron.2020.01.020_bib43","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1038\/gt.2014.101","article-title":"Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis","volume":"22","author":"Jackson","year":"2015","journal-title":"Gene Ther."},{"key":"10.1016\/j.neuron.2020.01.020_bib44","first-page":"e50245","article-title":"Purification of transcripts and metabolites from Drosophila heads","volume":"73","author":"Jensen","year":"2013","journal-title":"J.\u00a0Vis. Exp."},{"key":"10.1016\/j.neuron.2020.01.020_bib45","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1038\/nn.4085","article-title":"Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD\/ALS","volume":"18","author":"Jovi\u010di\u0107","year":"2015","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib46","doi-asserted-by":"crossref","first-page":"e1001052","DOI":"10.1371\/journal.pbio.1001052","article-title":"A yeast model of FUS\/TLS-dependent cytotoxicity","volume":"9","author":"Ju","year":"2011","journal-title":"PLoS Biol."},{"key":"10.1016\/j.neuron.2020.01.020_bib47","doi-asserted-by":"crossref","first-page":"E11904","DOI":"10.1073\/pnas.1810413115","article-title":"ALS mutations of FUS suppress protein translation and disrupt the regulation of nonsense-mediated decay","volume":"115","author":"Kamelgarn","year":"2018","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.01.020_bib48","doi-asserted-by":"crossref","first-page":"1803","DOI":"10.1093\/hmg\/ddw052","article-title":"Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation","volume":"25","author":"Kanekura","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.01.020_bib49","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1101\/gad.1389006","article-title":"Binding of a novel SMG-1-Upf1-eRF1-eRF3 complex (SURF) to the exon junction complex triggers Upf1 phosphorylation and nonsense-mediated mRNA decay","volume":"20","author":"Kashima","year":"2006","journal-title":"Genes Dev."},{"key":"10.1016\/j.neuron.2020.01.020_bib50","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1074\/mcp.M112.019414","article-title":"Identification of cargo proteins specific for the nucleocytoplasmic transport carrier transportin by combination of an in\u00a0vitro transport system and stable isotope labeling by amino acids in cell culture (SILAC)-based quantitative proteomics","volume":"12","author":"Kimura","year":"2013","journal-title":"Mol. Cell. Proteomics"},{"key":"10.1016\/j.neuron.2020.01.020_bib51","doi-asserted-by":"crossref","first-page":"24540","DOI":"10.1074\/jbc.M113.489286","article-title":"Identification of cargo proteins specific for importin-\u03b2 with importin-\u03b1 applying a stable isotope labeling by amino acids in cell culture (SILAC)-based in\u00a0vitro transport system","volume":"288","author":"Kimura","year":"2013","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.neuron.2020.01.020_bib52","doi-asserted-by":"crossref","first-page":"6","DOI":"10.7554\/eLife.21184","article-title":"Extensive cargo identification reveals distinct biological roles of the 12 importin pathways","volume":"6","author":"Kimura","year":"2017","journal-title":"eLife"},{"key":"10.1016\/j.neuron.2020.01.020_bib53","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1093\/emboj\/cdg017","article-title":"Termination of translation: interplay of mRNA, rRNAs and release factors?","volume":"22","author":"Kisselev","year":"2003","journal-title":"EMBO J."},{"key":"10.1016\/j.neuron.2020.01.020_bib54","doi-asserted-by":"crossref","first-page":"4573","DOI":"10.1093\/nar\/gkt113","article-title":"Two-step model of stop codon recognition by eukaryotic release factor eRF1","volume":"41","author":"Kryuchkova","year":"2013","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib55","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1242\/jcs.181008","article-title":"Nonsense-mediated mRNA decay in humans at a glance","volume":"129","author":"Kurosaki","year":"2016","journal-title":"J.\u00a0Cell Sci."},{"key":"10.1016\/j.neuron.2020.01.020_bib56","doi-asserted-by":"crossref","first-page":"1900","DOI":"10.1101\/gad.245506.114","article-title":"A post-translational regulatory switch on UPF1 controls targeted mRNA degradation","volume":"28","author":"Kurosaki","year":"2014","journal-title":"Genes Dev."},{"key":"10.1016\/j.neuron.2020.01.020_bib57","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1126\/science.1254917","article-title":"Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells","volume":"345","author":"Kwon","year":"2014","journal-title":"Science"},{"key":"10.1016\/j.neuron.2020.01.020_bib58","doi-asserted-by":"crossref","first-page":"1488","DOI":"10.1038\/nn.3230","article-title":"Divergent roles of ALS-linked proteins FUS\/TLS and TDP-43 intersect in processing long pre-mRNAs","volume":"15","author":"Lagier-Tourenne","year":"2012","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib59","doi-asserted-by":"crossref","first-page":"2510","DOI":"10.1093\/hmg\/ddr150","article-title":"A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43","volume":"20","author":"Lanson","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.01.020_bib60","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1016\/j.cell.2016.10.002","article-title":"C9orf72 dipeptide repeats impair the assembly, dynamics, and function of membrane-less organelles","volume":"167","author":"Lee","year":"2016","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib61","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1002\/jcb.24681","article-title":"Nuclear structures surrounding internal lamin invaginations","volume":"115","author":"Legartov\u00e1","year":"2014","journal-title":"J.\u00a0Cell. Biochem."},{"key":"10.1016\/j.neuron.2020.01.020_bib62","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1016\/j.cell.2016.10.003","article-title":"Toxic PR poly-dipeptides encoded by the C9orf72 repeat expansion target LC domain polymers","volume":"167","author":"Lin","year":"2016","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib63","doi-asserted-by":"crossref","first-page":"1425","DOI":"10.1038\/s41467-017-01757-0","article-title":"Origin of the omnipotence of eukaryotic release factor 1","volume":"8","author":"Lind","year":"2017","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.01.020_bib64","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.neuron.2016.09.015","article-title":"Poly(GR) in C9ORF72-related ALS\/FTD compromises mitochondrial function and increases oxidative stress and DNA damage in iPSC-derived motor neurons","volume":"92","author":"Lopez-Gonzalez","year":"2016","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib65","doi-asserted-by":"crossref","first-page":"7326","DOI":"10.1073\/pnas.1201724109","article-title":"Self-regulated viscous channel in the nuclear pore complex","volume":"109","author":"Ma","year":"2012","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.01.020_bib66","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1007\/s00401-015-1476-2","article-title":"Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers","volume":"130","author":"Mackenzie","year":"2015","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.neuron.2020.01.020_bib67","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.tcb.2011.03.008","article-title":"The nucleoplasmic reticulum: form and function","volume":"21","author":"Malhas","year":"2011","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.neuron.2020.01.020_bib68","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.neuron.2019.01.048","article-title":"RNA binding antagonizes neurotoxic phase transitions of TDP-43","volume":"102","author":"Mann","year":"2019","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib69","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1007\/s00401-014-1329-4","article-title":"C9orf72 FTLD\/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration","volume":"128","author":"May","year":"2014","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.neuron.2020.01.020_bib70","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1126\/science.1256800","article-title":"C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins","volume":"345","author":"Mizielinska","year":"2014","journal-title":"Science"},{"key":"10.1016\/j.neuron.2020.01.020_bib71","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1126\/science.1232927","article-title":"The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD\/ALS","volume":"339","author":"Mori","year":"2013","journal-title":"Science"},{"key":"10.1016\/j.neuron.2020.01.020_bib72","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1007\/s00401-013-1088-7","article-title":"hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive\/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations","volume":"125","author":"Mori","year":"2013","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.neuron.2020.01.020_bib73","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1126\/science.1134108","article-title":"Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"314","author":"Neumann","year":"2006","journal-title":"Science"},{"issue":"61","key":"10.1016\/j.neuron.2020.01.020_bib74","first-page":"3795","article-title":"Methods to assay Drosophila behavior","author":"Nichols","year":"2012","journal-title":"J.\u00a0Vis. Exp."},{"key":"10.1016\/j.neuron.2020.01.020_bib75","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1038\/nm.3620","article-title":"Intracellular calcium regulates nonsense-mediated mRNA decay","volume":"20","author":"Nickless","year":"2014","journal-title":"Nat. Med."},{"key":"10.1016\/j.neuron.2020.01.020_bib76","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1038\/nature05853","article-title":"HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS","volume":"447","author":"Pandey","year":"2007","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib77","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1021\/pr025556v","article-title":"Evaluation of multidimensional chromatography coupled with tandem mass spectrometry (LC\/LC-MS\/MS) for large-scale protein analysis: the yeast proteome","volume":"2","author":"Peng","year":"2003","journal-title":"J.\u00a0Proteome Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib78","doi-asserted-by":"crossref","DOI":"10.1074\/mcp.M110.007039","article-title":"Large-scale label-free quantitative proteomics of the pea aphid-Buchnera symbiosis","volume":"10","author":"Poliakov","year":"2011","journal-title":"Mol. Cell. Proteomics"},{"key":"10.1016\/j.neuron.2020.01.020_bib79","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1038\/nn.2779","article-title":"Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43","volume":"14","author":"Polymenidou","year":"2011","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib80","doi-asserted-by":"crossref","first-page":"1175","DOI":"10.1038\/nn.4065","article-title":"Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS","volume":"18","author":"Prudencio","year":"2015","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib81","doi-asserted-by":"crossref","first-page":"1504","DOI":"10.15252\/embj.201593791","article-title":"Minor intron splicing is regulated by FUS and affected by ALS-associated FUS mutants","volume":"35","author":"Reber","year":"2016","journal-title":"EMBO J."},{"key":"10.1016\/j.neuron.2020.01.020_bib82","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.neuron.2011.09.010","article-title":"A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD","volume":"72","author":"Renton","year":"2011","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib83","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1126\/science.1217421","article-title":"Extremely long-lived nuclear pore proteins in the rat brain","volume":"335","author":"Savas","year":"2012","journal-title":"Science"},{"key":"10.1016\/j.neuron.2020.01.020_bib84","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1038\/nmeth.1314","article-title":"SUnSET, a nonradioactive method to monitor protein synthesis","volume":"6","author":"Schmidt","year":"2009","journal-title":"Nat. Methods"},{"key":"10.1016\/j.neuron.2020.01.020_bib85","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1038\/nprot.2008.73","article-title":"Analyzing real-time PCR data by the comparative C(T) method","volume":"3","author":"Schmittgen","year":"2008","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.neuron.2020.01.020_bib86","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1080\/19491034.2017.1337621","article-title":"Nanoscale invaginations of the nuclear envelope: Shedding new light on wormholes with elusive function","volume":"8","author":"Schoen","year":"2017","journal-title":"Nucleus"},{"key":"10.1016\/j.neuron.2020.01.020_bib87","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/nm.4490","article-title":"Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS\/FTD human induced motor neurons","volume":"24","author":"Shi","year":"2018","journal-title":"Nat. Med."},{"key":"10.1016\/j.neuron.2020.01.020_bib88","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1016\/S0092-8674(00)80667-4","article-title":"The crystal structure of human eukaryotic release factor eRF1\u2014mechanism of stop codon recognition and peptidyl-tRNA hydrolysis","volume":"100","author":"Song","year":"2000","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib89","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/j.neuron.2014.09.019","article-title":"Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD\/ALS","volume":"84","author":"Su","year":"2014","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib90","doi-asserted-by":"crossref","first-page":"4234","DOI":"10.1073\/pnas.1716617115","article-title":"Intron retention induced by microsatellite expansions as a disease biomarker","volume":"115","author":"Sznajder","year":"2018","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.01.020_bib91","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1021\/pr015504q","article-title":"DTASelect and Contrast: tools for assembling and comparing protein identifications from shotgun proteomics","volume":"1","author":"Tabb","year":"2002","journal-title":"J.\u00a0Proteome Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib92","doi-asserted-by":"crossref","first-page":"2845","DOI":"10.1038\/s41467-018-05049-z","article-title":"Abnormal RNA stability in amyotrophic lateral sclerosis","volume":"9","author":"Tank","year":"2018","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.01.020_bib93","doi-asserted-by":"crossref","first-page":"2426","DOI":"10.1093\/hmg\/ddv005","article-title":"Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity","volume":"24","author":"Tao","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.01.020_bib94","doi-asserted-by":"crossref","first-page":"1785","DOI":"10.18632\/oncotarget.22751","article-title":"Comparative proteomics as a tool for identifying specific alterations within interferon response pathways in human glioblastoma multiforme cells","volume":"9","author":"Tarasova","year":"2017","journal-title":"Oncotarget"},{"key":"10.1016\/j.neuron.2020.01.020_bib95","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/nature20413","article-title":"Decoding ALS: from genes to mechanism","volume":"539","author":"Taylor","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib96","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1074\/mcp.M112.024877","article-title":"Identification of CRM1-dependent nuclear export cargos using quantitative mass spectrometry","volume":"12","author":"Thakar","year":"2013","journal-title":"Mol. Cell. Proteomics"},{"key":"10.1016\/j.neuron.2020.01.020_bib97","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/j.cell.2013.07.037","article-title":"Identification of long-lived proteins reveals exceptional stability of essential cellular structures","volume":"154","author":"Toyama","year":"2013","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib120","doi-asserted-by":"crossref","first-page":"9000","DOI":"10.1523\/JNEUROSCI.0118-17.2017","article-title":"Mutant Huntingtin Is Secreted via a Late Endosomal\/Lysosomal Unconventional Secretory Pathway","volume":"37","author":"Trajkovic","year":"2017","journal-title":"J. Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib98","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1101\/gad.209635.112","article-title":"Temporal and spatial characterization of nonsense-mediated mRNA decay","volume":"27","author":"Trcek","year":"2013","journal-title":"Genes Dev."},{"key":"10.1016\/j.neuron.2020.01.020_bib119","doi-asserted-by":"crossref","first-page":"D204","DOI":"10.1093\/nar\/gku989","article-title":"UniProt: a hub for protein information","volume":"43","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.neuron.2020.01.020_bib99","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1007\/s00401-015-1480-6","article-title":"Novel clinical associations with specific C9ORF72 transcripts in patients with repeat expansions in C9ORF72","volume":"130","author":"van Blitterswijk","year":"2015","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.neuron.2020.01.020_bib121","doi-asserted-by":"crossref","first-page":"3733","DOI":"10.1093\/hmg\/ddu081","article-title":"The histone deacetylase HDAC3 Is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1","volume":"23","author":"Venkatraman","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.01.020_bib100","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.tins.2014.05.006","article-title":"Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies","volume":"37","author":"Vucic","year":"2014","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.neuron.2020.01.020_bib101","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1016\/j.neuron.2014.12.010","article-title":"Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS\/FTD form toxic nuclear aggregates that initiate in\u00a0vitro and in\u00a0vivo neuronal death","volume":"84","author":"Wen","year":"2014","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.01.020_bib102","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/978-3-319-89689-2_5","article-title":"RNA degradation in neurodegenerative disease","volume":"20","author":"Weskamp","year":"2018","journal-title":"Adv. Neurobiol."},{"key":"10.1016\/j.neuron.2020.01.020_bib103","doi-asserted-by":"crossref","first-page":"11","DOI":"10.15252\/emmm.201809423","article-title":"Repeat-associated non-AUG translation in C9orf72-ALS\/FTD is driven by neuronal excitation and stress","volume":"11","author":"Westergard","year":"2019","journal-title":"EMBO Mol. Med."},{"key":"10.1016\/j.neuron.2020.01.020_bib104","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1126\/science.aad2033","article-title":"Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA","volume":"351","author":"Woerner","year":"2016","journal-title":"Science"},{"key":"10.1016\/j.neuron.2020.01.020_bib105","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1126\/science.aax2957","article-title":"Cellular RNA surveillance in health and disease","volume":"366","author":"Wolin","year":"2019","journal-title":"Science"},{"key":"10.1016\/j.neuron.2020.01.020_bib106","doi-asserted-by":"crossref","first-page":"5467","DOI":"10.1038\/ncomms6467","article-title":"TRF2 and lamin A\/C interact to facilitate the functional organization of chromosome ends","volume":"5","author":"Wood","year":"2014","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.01.020_bib107","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.jprot.2015.07.001","article-title":"ProLuCID, a fast and sensitive tandem mass spectra-based protein identification program","volume":"129","author":"Xu","year":"2006","journal-title":"J.\u00a0Proteomics"},{"key":"10.1016\/j.neuron.2020.01.020_bib108","doi-asserted-by":"crossref","first-page":"7778","DOI":"10.1073\/pnas.1219643110","article-title":"Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration","volume":"110","author":"Xu","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.01.020_bib109","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1093\/brain\/awz070","article-title":"Reactivation of nonsense-mediated mRNA decay protects against C9orf72 dipeptide-repeat neurotoxicity","volume":"142","author":"Xu","year":"2019","journal-title":"Brain"},{"key":"10.1016\/j.neuron.2020.01.020_bib110","doi-asserted-by":"crossref","first-page":"2244","DOI":"10.1016\/j.celrep.2017.05.056","article-title":"Evidence that C9ORF72 dipeptide repeat proteins associate with U2 snRNP to cause mis-splicing in ALS\/FTD patients","volume":"19","author":"Yin","year":"2017","journal-title":"Cell Rep."},{"key":"10.1016\/j.neuron.2020.01.020_bib111","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1186\/1741-7007-7-23","article-title":"Noisy splicing, more than expression regulation, explains why some exons are subject to nonsense-mediated mRNA decay","volume":"7","author":"Zhang","year":"2009","journal-title":"BMC Biol."},{"key":"10.1016\/j.neuron.2020.01.020_bib112","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1038\/nature14973","article-title":"The C9orf72 repeat expansion disrupts nucleocytoplasmic transport","volume":"525","author":"Zhang","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.01.020_bib113","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1080\/19491034.2016.1172152","article-title":"Nucleocytoplasmic transport in C9orf72-mediated ALS\/FTD","volume":"7","author":"Zhang","year":"2016","journal-title":"Nucleus"},{"key":"10.1016\/j.neuron.2020.01.020_bib114","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.1016\/j.ygeno.2018.07.008","article-title":"Characterization of proteins in different subcellular localizations for Escherichia coli K12","volume":"111","author":"Zhang","year":"2019","journal-title":"Genomics"},{"key":"10.1016\/j.neuron.2020.01.020_bib115","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1016\/j.stem.2018.02.012","article-title":"Dissecting the functional consequences of de novo DNA methylation dynamics in human motor neuron differentiation and physiology","volume":"22","author":"Ziller","year":"2018","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.neuron.2020.01.020_bib116","doi-asserted-by":"crossref","first-page":"E4968","DOI":"10.1073\/pnas.1315438110","article-title":"RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia","volume":"110","author":"Zu","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.01.020_bib117","doi-asserted-by":"crossref","first-page":"9873","DOI":"10.1128\/JVI.72.12.9873-9880.1998","article-title":"Self-inactivating lentivirus vector for safe and efficient in\u00a0vivo gene delivery","volume":"72","author":"Zufferey","year":"1998","journal-title":"J.\u00a0Virol."},{"key":"10.1016\/j.neuron.2020.01.020_bib118","doi-asserted-by":"crossref","first-page":"2339","DOI":"10.1021\/pr060161n","article-title":"Statistical analysis of membrane proteome expression changes in Saccharomyces cerevisiae","volume":"5","author":"Zybailov","year":"2006","journal-title":"J.\u00a0Proteome Res."}],"container-title":["Neuron"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896627320300465?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896627320300465?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T07:23:48Z","timestamp":1760253828000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0896627320300465"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4]]},"references-count":121,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,4]]}},"alternative-id":["S0896627320300465"],"URL":"https:\/\/doi.org\/10.1016\/j.neuron.2020.01.020","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/677419","asserted-by":"object"}]},"ISSN":["0896-6273"],"issn-type":[{"value":"0896-6273","type":"print"}],"subject":[],"published":{"date-parts":[[2020,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS\/FTD C9orf72 Toxicity","name":"articletitle","label":"Article Title"},{"value":"Neuron","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.neuron.2020.01.020","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Elsevier Inc.","name":"copyright","label":"Copyright"}]}}