{"doi":"10.1016/j.cell.2018.03.025","title":"Stress Granule Assembly Disrupts Nucleocytoplasmic Transport","abstract":null,"journal":"Cell","year":2018,"id":629551,"datarank":0.913356731317027,"base_score":6.089044875446846,"endowment":6.089044875446846,"self_citation_contribution":0.913356731317027,"citation_network_contribution":0.0,"self_endowment_contribution":0.913356731317027,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":440,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":569318,"name":"J. Gavin Daigle","orcid":null,"position":1,"is_corresponding":false},{"id":300981,"name":"Kathleen M. Cunningham","orcid":"0000-0002-1347-9087","position":2,"is_corresponding":false},{"id":254050,"name":"Alyssa N. Coyne","orcid":"0000-0002-3658-5325","position":3,"is_corresponding":false},{"id":300983,"name":"Kai Ruan","orcid":"0000-0002-3399-1862","position":4,"is_corresponding":false},{"id":303090,"name":"Jonathan C. Grima","orcid":null,"position":5,"is_corresponding":false},{"id":1630488,"name":"Kelly E. Bowen","orcid":null,"position":6,"is_corresponding":false},{"id":319823,"name":"Harsh Wadhwa","orcid":"0000-0003-4851-1902","position":7,"is_corresponding":false},{"id":283899,"name":"Peiguo Yang","orcid":"0000-0002-2334-5664","position":8,"is_corresponding":false},{"id":124979,"name":"Frank Rigo","orcid":null,"position":9,"is_corresponding":false},{"id":228281,"name":"J. Paul Taylor","orcid":"0000-0002-5794-0349","position":10,"is_corresponding":false},{"id":230690,"name":"Aaron D. Gitler","orcid":"0000-0001-8603-1526","position":11,"is_corresponding":false},{"id":229445,"name":"Jeffrey D. Rothstein","orcid":null,"position":12,"is_corresponding":false},{"id":257667,"name":"Thomas E. Lloyd","orcid":"0000-0003-4756-3700","position":13,"is_corresponding":false},{"id":1169312,"name":"Ke Zhang","orcid":"0000-0003-0209-2327","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Stress Granule Assembly Disrupts Nucleocytoplasmic Transport","abstract":"Defects in nucleocytoplasmic transport have been identified as a key pathogenic event in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mediated by a GGGGCC hexanucleotide repeat expansion in C9ORF72, the most common genetic cause of ALS/FTD. Furthermore, nucleocytoplasmic transport disruption has also been implicated in other neurodegenerative diseases with protein aggregation, suggesting a shared mechanism by which protein stress disrupts nucleocytoplasmic transport. Here, we show that cellular stress disrupts nucleocytoplasmic transport by localizing critical nucleocytoplasmic transport factors into stress granules, RNA/protein complexes that play a crucial role in ALS pathogenesis. Importantly, inhibiting stress granule assembly, such as by knocking down Ataxin-2, suppresses nucleocytoplasmic transport defects as well as neurodegeneration in C9ORF72-mediated ALS/FTD. Our findings identify a link between stress granule assembly and nucleocytoplasmic transport, two fundamental cellular processes implicated in the pathogenesis of C9ORF72-mediated ALS/FTD and other neurodegenerative diseases.","is_dataset_classified":null,"base_score":6.089044875446846,"endowment":6.089044875446846,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"29628143","pmcid":"PMC6083872","openalex_id":"https://openalex.org/W2796205057","authors":[],"funders":[{"funder_name":"ALSA","grant_id":"NS100401","title":null},{"funder_name":"ALSA","grant_id":"NS103360","title":null},{"funder_name":"NIH","grant_id":"R01NS085207","title":null},{"funder_name":"NIH","grant_id":"R01NS099320","title":null},{"funder_name":"NIH","grant_id":"P01NS09911","title":null},{"funder_name":"NIH","grant_id":"R01NS094239","title":null},{"funder_name":"NIH","grant_id":"U24NS078736","title":null},{"funder_name":"NIH","grant_id":"R01NS082563","title":null},{"funder_name":"NIH","grant_id":"NS094239","title":null},{"funder_name":"NIH","grant_id":"P30NS050274","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"U54 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Association","grant_id":"","title":null},{"funder_name":"ALSA","grant_id":"","title":null}],"total_grants":26,"fwci":34.5778,"citation_percentile":0.99902681,"influential_citations":0,"citation_trend":[{"year":2018,"count":25},{"year":2019,"count":63},{"year":2020,"count":56},{"year":2021,"count":78},{"year":2022,"count":52},{"year":2023,"count":51},{"year":2024,"count":52},{"year":2025,"count":36},{"year":2026,"count":26}],"oa_status":"bronze","license":"http://www.elsevier.com/open-access/userlicense/1.0/","oa_locations":[{"url":"http://www.cell.com/article/S0092867418303052/pdf","host_type":"journal"},{"url":"http://www.cell.com/article/S0092867418303052/pdf","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S0092867418303052?httpAccept=text/xml","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S0092867418303052?httpAccept=text/plain","host_type":"publisher"},{"url":"https://doi.org/10.1016/j.cell.2018.03.025","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/29628143","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/6083872","host_type":"repository"}],"fields_of_study":["Amyotrophic Lateral Sclerosis Research","Neurogenetic and Muscular Disorders Research","RNA Research and Splicing","Active Transport, Cell Nucleus","Aged","Amyotrophic Lateral Sclerosis","Arsenites","Ataxin-2","C9orf72 Protein","DNA Repeat Expansion","Female","Frontotemporal Dementia","HEK293 Cells","Humans","Male","Membrane Glycoproteins","Middle Aged","Nuclear Pore Complex Proteins","Oxidative Stress","RNA Interference","RNA, Small Interfering","Sodium Compounds","alpha Karyopherins","beta Karyopherins","ran GTP-Binding Protein","Frontotemporal Dementia With Motor Neuron Disease"],"mesh_terms":["Ataxin-2","C9orf72 Protein","Aged","Amyotrophic Lateral Sclerosis","Female","Humans","Male","Membrane Glycoproteins","Middle Aged","Sodium Compounds","Arsenites","Oxidative Stress","ran GTP-Binding Protein","Active Transport, Cell Nucleus","Nuclear Pore Complex Proteins","alpha Karyopherins","beta Karyopherins","RNA Interference","RNA, Small Interfering","DNA Repeat Expansion","Frontotemporal Dementia","HEK293 Cells"],"keywords":["C9orf72","Stress granule","Neurodegeneration","Biology","Cell biology","Ran","Frontotemporal dementia","Nuclear transport","Amyotrophic lateral sclerosis","Transport protein","Trinucleotide repeat expansion","Cell nucleus","Nucleus","Genetics","Gene","Dementia","Messenger RNA","Medicine","Disease","ALS","Nucleocytoplasmic 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