{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T00:25:36Z","timestamp":1791851136057,"version":"4.3.4"},"reference-count":104,"publisher":"Oxford University Press (OUP)","issue":"16","license":[{"start":{"date-parts":[[2024,8,6]],"date-time":"2024-08-06T00:00:00Z","timestamp":1722902400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P50HD104463"],"award-info":[{"award-number":["P50HD104463"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01NS099280"],"award-info":[{"award-number":["R01NS099280"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01NS086810"],"award-info":[{"award-number":["R01NS086810"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 NS097542"],"award-info":[{"award-number":["R01 NS097542"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 NS113943"],"award-info":[{"award-number":["R01 NS113943"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R56 NS128110"],"award-info":[{"award-number":["R56 NS128110"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["F31NS115257"],"award-info":[{"award-number":["F31NS115257"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIH T-32-NS076401"],"award-info":[{"award-number":["NIH T-32-NS076401"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ann Arbor Active Against ALS"},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"publisher","award":["2018-03843"],"award-info":[{"award-number":["2018-03843"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000738","name":"Veterans Affairs","doi-asserted-by":"publisher","award":["BLRD BX004842"],"award-info":[{"award-number":["BLRD BX004842"]}],"id":[{"id":"10.13039\/100000738","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Cellular stress pathways that inhibit translation initiation lead to transient formation of cytoplasmic RNA\/protein complexes known as stress granules. Many of the proteins found within stress granules and the dynamics of stress granule formation and dissolution are implicated in neurodegenerative disease. Whether stress granule formation is protective or harmful in neurodegenerative conditions is not known. To address this, we took advantage of the alphavirus protein nsP3, which selectively binds dimers of the central stress granule nucleator protein G3BP and markedly reduces stress granule formation without directly impacting the protein translational inhibitory pathways that trigger stress granule formation. In Drosophila and rodent neurons, reducing stress granule formation with nsP3 had modest impacts on lifespan even in the setting of serial stress pathway induction. In contrast, reducing stress granule formation in models of ataxia, amyotrophic lateral sclerosis and frontotemporal dementia largely exacerbated disease phenotypes. These data support a model whereby stress granules mitigate, rather than promote, neurodegenerative cascades.<\/jats:p>","DOI":"10.1093\/nar\/gkae655","type":"journal-article","created":{"date-parts":[[2024,7,17]],"date-time":"2024-07-17T01:08:23Z","timestamp":1721178503000},"page":"9745-9759","source":"Crossref","is-referenced-by-count":36,"title":["Stress granule formation helps to mitigate neurodegeneration"],"prefix":"10.1093","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8615-2894","authenticated-orcid":false,"given":"M\u00a0Rebecca","family":"Glineburg","sequence":"first","affiliation":[{"name":"Biological Sciences, Schmid College of Science and Technology, Chapman University , 1 University Drive , Orange , CA 92866 , USA"},{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evrim","family":"Yildirim","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Gomez","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"},{"name":"Cell and Molecular Biology Graduate Program, University of Michigan , Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genesis","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"},{"name":"Neuroscience Graduate Program, University of Michigan , Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaclyn","family":"Pak","sequence":"additional","affiliation":[{"name":"Biological Sciences, Schmid College of Science and Technology, Chapman University , 1 University Drive , Orange , CA 92866 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xingli","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Altheim","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob","family":"Waksmacki","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald\u00a0M","family":"McInerney","sequence":"additional","affiliation":[{"name":"Department of Microbiology, Tumor and Cell Biology, Karolinska Institutet , Stockholm 17165 , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sami\u00a0J","family":"Barmada","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4781-6376","authenticated-orcid":false,"given":"Peter\u00a0K","family":"Todd","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , 109 Zina Pitcher Place,\u00a0BSRB48109-2200 , Ann Arbor , MI 4005, USA"},{"name":"Veterans Affairs Medical Center , Ann Arbor , MI , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,8,6]]},"reference":[{"key":"2024090904023425600_B1","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1126\/science.1209038","article-title":"The unfolded protein response: from stress pathway to homeostatic regulation","volume":"334","author":"Walter","year":"2011","journal-title":"Science"},{"key":"2024090904023425600_B2","doi-asserted-by":"crossref","first-page":"aad3867","DOI":"10.1126\/science.aad3867","article-title":"Translation from the 5\u2032 untranslated region shapes the integrated stress response","volume":"351","author":"Starck","year":"2016","journal-title":"Science"},{"key":"2024090904023425600_B3","doi-asserted-by":"crossref","first-page":"1257","DOI":"10.1083\/jcb.151.6.1257","article-title":"Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules","volume":"151","author":"Kedersha","year":"2000","journal-title":"J. Cell Biol."},{"key":"2024090904023425600_B4","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1083\/jcb.200212128","article-title":"The RasGAP-associated endoribonuclease G3BP assembles stress granules","volume":"160","author":"Tourriere","year":"2003","journal-title":"J. Cell Biol."},{"key":"2024090904023425600_B5","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1083\/jcb.201508028","article-title":"G3BP-Caprin1-USP10 complexes mediate stress granule condensation and associate with 40S subunits","volume":"212","author":"Kedersha","year":"2016","journal-title":"J. Cell Biol."},{"key":"2024090904023425600_B6","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/j.cell.2020.03.046","article-title":"G3BP1 is a tunable switch that triggers phase separation to assemble stress granules","volume":"181","author":"Yang","year":"2020","journal-title":"Cell"},{"key":"2024090904023425600_B7","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1083\/jcb.147.7.1431","article-title":"RNA-binding proteins TIA-1 and TIAR link the phosphorylation of eIF-2 alpha to the assembly of mammalian stress granules","volume":"147","author":"Kedersha","year":"1999","journal-title":"J. Cell Biol."},{"key":"2024090904023425600_B8","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1016\/j.molcel.2009.11.020","article-title":"Eukaryotic stress granules: the ins and outs of translation","volume":"36","author":"Buchan","year":"2009","journal-title":"Mol. Cell"},{"key":"2024090904023425600_B9","doi-asserted-by":"crossref","first-page":"104639","DOI":"10.1016\/j.nbd.2019.104639","article-title":"Stress granule mediated protein aggregation and underlying gene defects in the FTD-ALS spectrum","volume":"134","author":"Baradaran-Heravi","year":"2020","journal-title":"Neurobiol. Dis."},{"key":"2024090904023425600_B10","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.neuron.2013.07.033","article-title":"Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis","volume":"79","author":"Ling","year":"2013","journal-title":"Neuron"},{"key":"2024090904023425600_B11","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1038\/s41583-019-0222-5","article-title":"Stress granules and neurodegeneration","volume":"20","author":"Wolozin","year":"2019","journal-title":"Nat. Rev. Neurosci."},{"key":"2024090904023425600_B12","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1523\/JNEUROSCI.4988-09.2010","article-title":"Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis","volume":"30","author":"Barmada","year":"2010","journal-title":"J. Neurosci."},{"key":"2024090904023425600_B13","doi-asserted-by":"crossref","first-page":"2339","DOI":"10.4161\/cc.25705","article-title":"Unpicking neurodegeneration in a dish with human pluripotent stem cells: one cell type at a time","volume":"12","author":"Bilican","year":"2013","journal-title":"Cell Cycle"},{"key":"2024090904023425600_B14","doi-asserted-by":"crossref","first-page":"4697","DOI":"10.1073\/pnas.1300398110","article-title":"Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy","volume":"110","author":"Serio","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024090904023425600_B15","doi-asserted-by":"crossref","first-page":"6439","DOI":"10.1073\/pnas.0802082105","article-title":"A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity","volume":"105","author":"Johnson","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024090904023425600_B16","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","article-title":"TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"351","author":"Arai","year":"2006","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"2024090904023425600_B17","doi-asserted-by":"crossref","first-page":"802","DOI":"10.1016\/j.neuron.2019.05.048","article-title":"Small-molecule modulation of TDP-43 recruitment to stress granules prevents persistent TDP-43 accumulation in ALS\/FTD","volume":"103","author":"Fang","year":"2019","journal-title":"Neuron"},{"key":"2024090904023425600_B18","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1111\/j.1471-4159.2009.06383.x","article-title":"TDP-43 is recruited to stress granules in conditions of oxidative insult","volume":"111","author":"Colombrita","year":"2009","journal-title":"J. Neurochem."},{"key":"2024090904023425600_B19","doi-asserted-by":"crossref","first-page":"611601","DOI":"10.3389\/fcell.2021.611601","article-title":"TDP-43 mutation affects stress granule dynamics in differentiated NSC-34 motoneuron-like cells","volume":"9","author":"Ding","year":"2021","journal-title":"Front. Cell Dev. Biol."},{"key":"2024090904023425600_B20","doi-asserted-by":"crossref","first-page":"105050","DOI":"10.1016\/j.nbd.2020.105050","article-title":"An ALS-linked mutation in TDP-43 disrupts normal protein interactions in the motor neuron response to oxidative stress","volume":"144","author":"Feneberg","year":"2020","journal-title":"Neurobiol. Dis."},{"key":"2024090904023425600_B21","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":"2024090904023425600_B22","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":"2024090904023425600_B23","doi-asserted-by":"crossref","first-page":"2005","DOI":"10.1038\/s41467-017-02200-0","article-title":"RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response","volume":"8","author":"Green","year":"2017","journal-title":"Nat. Commun."},{"key":"2024090904023425600_B24","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":"2024090904023425600_B25","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":"2024090904023425600_B26","doi-asserted-by":"crossref","first-page":"1630","DOI":"10.1093\/hmg\/ddu576","article-title":"Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD\/ALS","volume":"24","author":"Yamakawa","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"2024090904023425600_B27","doi-asserted-by":"crossref","first-page":"e0165084","DOI":"10.1371\/journal.pone.0165084","article-title":"Distinct C9orf72-associated dipeptide repeat structures correlate with neuronal toxicity","volume":"11","author":"Flores","year":"2016","journal-title":"PLoS One"},{"key":"2024090904023425600_B28","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/j.nbd.2018.05.009","article-title":"Translation of dipeptide repeat proteins from the C9ORF72 expanded repeat is associated with cellular stress","volume":"116","author":"Sonobe","year":"2018","journal-title":"Neurobiol. Dis."},{"key":"2024090904023425600_B29","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1038\/s41467-017-02495-z","article-title":"C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2alpha phosphorylation","volume":"9","author":"Cheng","year":"2018","journal-title":"Nat. Commun."},{"key":"2024090904023425600_B30","doi-asserted-by":"crossref","first-page":"100914","DOI":"10.1016\/j.jbc.2021.100914","article-title":"The RNA helicase DHX36-G4R1 modulates C9orf72 GGGGCC hexanucleotide repeat-associated translation","volume":"297","author":"Tseng","year":"2021","journal-title":"J. Biol. Chem."},{"key":"2024090904023425600_B31","doi-asserted-by":"crossref","first-page":"e9423","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":"2024090904023425600_B32","doi-asserted-by":"crossref","first-page":"105051","DOI":"10.1016\/j.nbd.2020.105051","article-title":"Chronic stress induces formation of stress granules and pathological TDP-43 aggregates in human ALS fibroblasts and iPSC-motoneurons","volume":"145","author":"Ratti","year":"2020","journal-title":"Neurobiol. Dis."},{"key":"2024090904023425600_B33","doi-asserted-by":"crossref","first-page":"e39578","DOI":"10.7554\/eLife.39578","article-title":"Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology","volume":"8","author":"Zhang","year":"2019","journal-title":"eLife"},{"key":"2024090904023425600_B34","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1126\/science.aaa4484","article-title":"Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit","volume":"348","author":"Das","year":"2015","journal-title":"Science"},{"key":"2024090904023425600_B35","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1038\/nn.2297","article-title":"A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice","volume":"12","author":"Saxena","year":"2009","journal-title":"Nat. Neurosci."},{"key":"2024090904023425600_B36","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1038\/ng.2853","article-title":"Therapeutic modulation of eIF2alpha phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models","volume":"46","author":"Kim","year":"2014","journal-title":"Nat. Genet."},{"key":"2024090904023425600_B37","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1007\/s00401-015-1487-z","article-title":"PERK inhibition prevents tau-mediated neurodegeneration in a mouse model of frontotemporal dementia","volume":"130","author":"Radford","year":"2015","journal-title":"Acta Neuropathol."},{"key":"2024090904023425600_B38","doi-asserted-by":"crossref","first-page":"10460","DOI":"10.1073\/pnas.0504249102","article-title":"Stimulation of mammalian translation initiation factor eIF4A activity by a small molecule inhibitor of eukaryotic translation","volume":"102","author":"Bordeleau","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024090904023425600_B39","doi-asserted-by":"crossref","first-page":"32870","DOI":"10.1074\/jbc.M606149200","article-title":"Eukaryotic initiation factor 2alpha-independent pathway of stress granule induction by the natural product pateamine A","volume":"281","author":"Dang","year":"2006","journal-title":"J. Biol. Chem."},{"key":"2024090904023425600_B40","doi-asserted-by":"crossref","first-page":"160078","DOI":"10.1098\/rsob.160078","article-title":"Combined structural, biochemical and cellular evidence demonstrates that both FGDF motifs in alphavirus nsP3 are required for efficient replication","volume":"6","author":"Schulte","year":"2016","journal-title":"Open Biol."},{"key":"2024090904023425600_B41","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1089\/dna.2015.2957","article-title":"FGDF motif regulation of stress granule formation","volume":"34","author":"McInerney","year":"2015","journal-title":"DNA Cell Biol."},{"key":"2024090904023425600_B42","doi-asserted-by":"crossref","first-page":"e1004659","DOI":"10.1371\/journal.ppat.1004659","article-title":"Viral and cellular proteins containing FGDF motifs bind G3BP to block stress granule formation","volume":"11","author":"Panas","year":"2015","journal-title":"PLoS Pathog."},{"key":"2024090904023425600_B43","doi-asserted-by":"crossref","first-page":"5888","DOI":"10.1128\/JVI.00439-14","article-title":"The C-terminal repeat domains of nsP3 from the Old World alphaviruses bind directly to G3BP","volume":"88","author":"Panas","year":"2014","journal-title":"J. Virol."},{"key":"2024090904023425600_B44","doi-asserted-by":"crossref","first-page":"4701","DOI":"10.1091\/mbc.e12-08-0619","article-title":"Sequestration of G3BP coupled with efficient translation inhibits stress granules in Semliki Forest virus infection","volume":"23","author":"Panas","year":"2012","journal-title":"Mol. Biol. Cell"},{"key":"2024090904023425600_B45","doi-asserted-by":"crossref","first-page":"e1007842","DOI":"10.1371\/journal.ppat.1007842","article-title":"Separate domains of G3BP promote efficient clustering of alphavirus replication complexes and recruitment of the translation initiation machinery","volume":"15","author":"Gotte","year":"2019","journal-title":"PLoS Pathog."},{"key":"2024090904023425600_B46","doi-asserted-by":"crossref","first-page":"e01681-19","DOI":"10.1128\/JVI.01681-19","article-title":"Sensitivity of alphaviruses to G3BP deletion correlates with efficiency of replicase polyprotein processing","volume":"94","author":"Gotte","year":"2020","journal-title":"J. Virol."},{"key":"2024090904023425600_B47","doi-asserted-by":"crossref","first-page":"3312","DOI":"10.1073\/pnas.0611511104","article-title":"An optimized transgenesis system for Drosophila using germ-line-specific phiC31 integrases","volume":"104","author":"Bischof","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024090904023425600_B48","doi-asserted-by":"crossref","first-page":"109376","DOI":"10.1016\/j.celrep.2021.109376","article-title":"Human oncoprotein 5MP suppresses general and repeat-associated non-AUG translation via eIF3 by a common mechanism","volume":"36","author":"Singh","year":"2021","journal-title":"Cell Rep."},{"key":"2024090904023425600_B49","doi-asserted-by":"crossref","first-page":"4606","DOI":"10.1038\/s41598-018-22858-w","article-title":"TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia","volume":"8","author":"Archbold","year":"2018","journal-title":"Sci. Rep."},{"key":"2024090904023425600_B50","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1016\/j.celrep.2019.03.093","article-title":"An Intramolecular Salt Bridge Linking TDP43 RNA Binding, Protein Stability, and TDP43-Dependent Neurodegeneration","volume":"27","author":"Flores","year":"2019","journal-title":"Cell Rep."},{"key":"2024090904023425600_B51","doi-asserted-by":"crossref","first-page":"e35977","DOI":"10.7554\/eLife.35977","article-title":"Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization","volume":"7","author":"Malik","year":"2018","journal-title":"eLife"},{"key":"2024090904023425600_B52","doi-asserted-by":"crossref","DOI":"10.3791\/59036-v","article-title":"Monitoring neuronal survival via longitudinal fluorescence microscopy","author":"Weskamp","year":"2019","journal-title":"J. Vis. Exp."},{"key":"2024090904023425600_B53","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","article-title":"Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death","volume":"431","author":"Arrasate","year":"2004","journal-title":"Nature"},{"key":"2024090904023425600_B54","doi-asserted-by":"crossref","first-page":"7551","DOI":"10.1038\/s41598-018-25767-0","article-title":"TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types","volume":"8","author":"Khalfallah","year":"2018","journal-title":"Sci. Rep."},{"key":"2024090904023425600_B55","doi-asserted-by":"crossref","first-page":"E10495","DOI":"10.1073\/pnas.1810522115","article-title":"Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly","volume":"115","author":"Sharkey","year":"2018","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024090904023425600_B56","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1007\/978-1-0716-2597-2_34","article-title":"Neuronal puncta\/aggregate formation by WT and mutant UBQLN2","volume":"2551","author":"Safren","year":"2023","journal-title":"Methods Mol. Biol."},{"key":"2024090904023425600_B57","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. U.S.A."},{"key":"2024090904023425600_B58","doi-asserted-by":"crossref","first-page":"e47498","DOI":"10.15252\/embr.201847498","article-title":"DDX3X and specific initiation factors modulate FMR1 repeat-associated non-AUG-initiated translation","volume":"20","author":"Linsalata","year":"2019","journal-title":"EMBO Rep."},{"key":"2024090904023425600_B59","doi-asserted-by":"crossref","first-page":"625711","DOI":"10.3389\/fcell.2021.625711","article-title":"A tale of 20 alphaviruses; inter-species diversity and conserved interactions between viral non-structural protein 3 and stress granule proteins","volume":"9","author":"Nowee","year":"2021","journal-title":"Front. Cell Dev. Biol."},{"key":"2024090904023425600_B60","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1186\/s13071-015-1070-4","article-title":"Mosquito Rasputin interacts with chikungunya virus nsP3 and determines the infection rate in Aedes albopictus","volume":"8","author":"Fros","year":"2015","journal-title":"Parasit. Vectors"},{"key":"2024090904023425600_B61","doi-asserted-by":"crossref","first-page":"e12068","DOI":"10.7554\/eLife.12068","article-title":"A genome-wide resource for the analysis of protein localisation in Drosophila","volume":"5","author":"Sarov","year":"2016","journal-title":"eLife"},{"key":"2024090904023425600_B62","doi-asserted-by":"crossref","first-page":"3840","DOI":"10.1242\/dev.149807","article-title":"A fluorescent toolkit for spatiotemporal tracking of apoptotic cells in living Drosophila tissues","volume":"144","author":"Schott","year":"2017","journal-title":"Development"},{"key":"2024090904023425600_B63","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1093\/emboj\/19.4.589","article-title":"Induction of apoptosis by Drosophila reaper, hid and grim through inhibition of IAP function","volume":"19","author":"Goyal","year":"2000","journal-title":"EMBO J."},{"key":"2024090904023425600_B64","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1016\/j.gep.2009.01.002","article-title":"Genomic mapping and expression patterns of C380, OK6 and D42 enhancer trap lines in the larval nervous system of Drosophila","volume":"9","author":"Sanyal","year":"2009","journal-title":"Gene Expr. Patterns"},{"key":"2024090904023425600_B65","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":"2024090904023425600_B66","doi-asserted-by":"crossref","first-page":"e13250","DOI":"10.1371\/journal.pone.0013250","article-title":"Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue","volume":"5","author":"Liu-Yesucevitz","year":"2010","journal-title":"PLoS One"},{"key":"2024090904023425600_B67","doi-asserted-by":"crossref","first-page":"7729","DOI":"10.1523\/JNEUROSCI.5894-09.2010","article-title":"TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP\/p97","volume":"30","author":"Ritson","year":"2010","journal-title":"J. Neurosci."},{"key":"2024090904023425600_B68","doi-asserted-by":"crossref","first-page":"4317","DOI":"10.1093\/hmg\/ddv165","article-title":"RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome","volume":"24","author":"Oh","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"2024090904023425600_B69","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1126\/science.1154584","article-title":"TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis","volume":"319","author":"Sreedharan","year":"2008","journal-title":"Science"},{"key":"2024090904023425600_B70","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1186\/s40478-020-01002-8","article-title":"The carboxyl termini of RAN translated GGGGCC nucleotide repeat expansions modulate toxicity in models of ALS\/FTD","volume":"8","author":"He","year":"2020","journal-title":"Acta Neuropathol. Commun."},{"key":"2024090904023425600_B71","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1097\/WCO.0000000000000130","article-title":"C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia: gain or loss of function?","volume":"27","author":"Mizielinska","year":"2014","journal-title":"Curr. Opin. Neurol."},{"key":"2024090904023425600_B72","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1016\/j.neuron.2013.03.026","article-title":"CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome","volume":"78","author":"Todd","year":"2013","journal-title":"Neuron"},{"key":"2024090904023425600_B73","doi-asserted-by":"crossref","first-page":"e12247","DOI":"10.1371\/journal.pone.0012247","article-title":"TDP-43-mediated neuron loss in vivo requires RNA-binding activity","volume":"5","author":"Voigt","year":"2010","journal-title":"PLoS One"},{"key":"2024090904023425600_B74","doi-asserted-by":"crossref","first-page":"2346","DOI":"10.1093\/brain\/awac473","article-title":"The stress granule protein G3BP1 alleviates spinocerebellar ataxia-associated deficits","volume":"146","author":"Koppenol","year":"2023","journal-title":"Brain"},{"key":"2024090904023425600_B75","doi-asserted-by":"crossref","first-page":"3194","DOI":"10.4161\/cc.26241","article-title":"Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm","volume":"12","author":"Shelkovnikova","year":"2013","journal-title":"Cell Cycle"},{"key":"2024090904023425600_B76","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/j.molcel.2018.07.002","article-title":"Poly(ADP-ribose) prevents pathological phase separation of TDP-43 by promoting liquid demixing and stress granule localization","volume":"71","author":"McGurk","year":"2018","journal-title":"Mol. Cell"},{"key":"2024090904023425600_B77","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.neuron.2019.02.038","article-title":"Cytoplasmic TDP-43 de-mixing independent of stress granules drives inhibition of nuclear import, loss of nuclear TDP-43, and cell death","volume":"102","author":"Gasset-Rosa","year":"2019","journal-title":"Neuron"},{"key":"2024090904023425600_B78","doi-asserted-by":"crossref","first-page":"2841","DOI":"10.1038\/emboj.2010.143","article-title":"ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import","volume":"29","author":"Dormann","year":"2010","journal-title":"EMBO J."},{"key":"2024090904023425600_B79","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1016\/j.neuron.2017.07.025","article-title":"TIA1 mutations in amyotrophic lateral sclerosis and frontotemporal dementia promote phase separation and alter stress granule dynamics","volume":"95","author":"Mackenzie","year":"2017","journal-title":"Neuron"},{"key":"2024090904023425600_B80","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1097\/NEN.0000000000000102","article-title":"Poly-A binding protein-1 localization to a subset of TDP-43 inclusions in amyotrophic lateral sclerosis occurs more frequently in patients harboring an expansion in C9orf72","volume":"73","author":"McGurk","year":"2014","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"2024090904023425600_B81","doi-asserted-by":"crossref","first-page":"48","DOI":"10.15252\/emmm.201708202","article-title":"A proteomic network approach across the ALS-FTD disease spectrum resolves clinical phenotypes and genetic vulnerability in human brain","volume":"10","author":"Umoh","year":"2018","journal-title":"EMBO Mol. Med."},{"key":"2024090904023425600_B82","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.3390\/biom10101367","article-title":"Stress granule assembly can facilitate but is not required for TDP-43 cytoplasmic aggregation","volume":"10","author":"Fernandes","year":"2020","journal-title":"Biomolecules"},{"key":"2024090904023425600_B83","doi-asserted-by":"crossref","first-page":"8270","DOI":"10.1523\/JNEUROSCI.1592-12.2012","article-title":"Contrasting pathology of the stress granule proteins TIA-1 and G3BP in tauopathies","volume":"32","author":"Vanderweyde","year":"2012","journal-title":"J. Neurosci."},{"key":"2024090904023425600_B84","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1016\/j.bbrc.2007.01.043","article-title":"Activation of the unfolded protein response in Parkinson's disease","volume":"354","author":"Hoozemans","year":"2007","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"2024090904023425600_B85","doi-asserted-by":"crossref","first-page":"2429","DOI":"10.1097\/00001756-200212200-00011","article-title":"Phosphorylation of eukaryotic initiation factor-2alpha (eIF2alpha) is associated with neuronal degeneration in Alzheimer's disease","volume":"13","author":"Chang","year":"2002","journal-title":"Neuroreport"},{"key":"2024090904023425600_B86","doi-asserted-by":"crossref","first-page":"6896","DOI":"10.1007\/s12035-023-03509-2","article-title":"Amplifying the heat shock response ameliorates ALS and FTD pathology in mouse and human models","volume":"60","author":"Ahmed","year":"2023","journal-title":"Mol. Neurobiol."},{"key":"2024090904023425600_B87","doi-asserted-by":"crossref","first-page":"3111","DOI":"10.1093\/brain\/awm190","article-title":"Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis","volume":"130","author":"Ilieva","year":"2007","journal-title":"Brain"},{"key":"2024090904023425600_B88","doi-asserted-by":"crossref","first-page":"e140723","DOI":"10.1172\/JCI140723","article-title":"Huntington's disease mice and human brain tissue exhibit increased G3BP1 granules and TDP43 mislocalization","volume":"131","author":"Sanchez","year":"2021","journal-title":"J. Clin. Invest."},{"key":"2024090904023425600_B89","doi-asserted-by":"crossref","first-page":"e13136","DOI":"10.1111\/acel.13136","article-title":"The involvement of stress granules in aging and aging-associated diseases","volume":"19","author":"Cao","year":"2020","journal-title":"Aging Cell"},{"key":"2024090904023425600_B90","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1007\/s00401-014-1336-5","article-title":"Aggregation-prone c9FTD\/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress","volume":"128","author":"Zhang","year":"2014","journal-title":"Acta Neuropathol."},{"key":"2024090904023425600_B91","doi-asserted-by":"crossref","first-page":"1607","DOI":"10.1016\/j.neurobiolaging.2007.04.014","article-title":"Abeta inhibits the proteasome and enhances amyloid and tau accumulation","volume":"29","author":"Tseng","year":"2008","journal-title":"Neurobiol. Aging"},{"key":"2024090904023425600_B92","doi-asserted-by":"crossref","first-page":"469","DOI":"10.2174\/156720508785908883","article-title":"Increased Abeta1-42 production sensitizes neuroblastoma cells for ER stress toxicity","volume":"5","author":"Chafekar","year":"2008","journal-title":"Curr. Alzheimer Res."},{"key":"2024090904023425600_B93","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1111\/j.1471-4159.2010.07143.x","article-title":"Induction of the unfolded protein response by alpha-synuclein in experimental models of Parkinson's disease","volume":"116","author":"Bellucci","year":"2011","journal-title":"J. Neurochem."},{"key":"2024090904023425600_B94","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1159\/000334536","article-title":"Activation of the unfolded protein response is an early event in Alzheimer's and Parkinson's disease","volume":"10","author":"Hoozemans","year":"2012","journal-title":"Neurodegener. Dis."},{"key":"2024090904023425600_B95","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1038\/s43587-021-00112-9","article-title":"Perspective: modulating the integrated stress response to slow aging and ameliorate age-related pathology","volume":"1","author":"Derisbourg","year":"2021","journal-title":"Nat. Aging."},{"key":"2024090904023425600_B96","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/j.bbamcr.2016.12.008","article-title":"Dissecting the molecular mechanisms that impair stress granule formation in aging cells","volume":"1864","author":"Moujaber","year":"2017","journal-title":"Biochim. Biophys. Acta. Mol. Cell Res."},{"key":"2024090904023425600_B97","doi-asserted-by":"crossref","first-page":"e2788","DOI":"10.1038\/cddis.2017.199","article-title":"Chronically stressed or stress-preconditioned neurons fail to maintain stress granule assembly","volume":"8","author":"Shelkovnikova","year":"2017","journal-title":"Cell Death Dis."},{"key":"2024090904023425600_B98","doi-asserted-by":"crossref","first-page":"e06807","DOI":"10.7554\/eLife.06807","article-title":"Promiscuous interactions and protein disaggregases determine the material state of stress-inducible RNP granules","volume":"4","author":"Kroschwald","year":"2015","journal-title":"eLife"},{"key":"2024090904023425600_B99","doi-asserted-by":"crossref","first-page":"eabk2022","DOI":"10.1126\/sciadv.abk2022","article-title":"Temporal control of the integrated stress response by a stochastic molecular switch","volume":"8","author":"Klein","year":"2022","journal-title":"Sci. Adv."},{"key":"2024090904023425600_B100","doi-asserted-by":"crossref","first-page":"85","DOI":"10.3389\/fnmol.2017.00085","article-title":"Control of mRNA translation in ALS proteinopathy","volume":"10","author":"Cestra","year":"2017","journal-title":"Front. Mol. Neurosci."},{"key":"2024090904023425600_B101","doi-asserted-by":"crossref","first-page":"1008","DOI":"10.1093\/hmg\/ddq546","article-title":"The unfolded protein response in familial amyotrophic lateral sclerosis","volume":"20","author":"Wang","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"2024090904023425600_B102","doi-asserted-by":"crossref","first-page":"2629","DOI":"10.1093\/hmg\/ddt658","article-title":"An enhanced integrated stress response ameliorates mutant SOD1-induced ALS","volume":"23","author":"Wang","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"2024090904023425600_B103","doi-asserted-by":"crossref","first-page":"104702","DOI":"10.1016\/j.nbd.2019.104702","article-title":"Knockdown of GADD34 in neonatal mutant SOD1 mice ameliorates ALS","volume":"136","author":"Ghadge","year":"2020","journal-title":"Neurobiol. Dis."},{"key":"2024090904023425600_B104","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1111\/bph.16260","article-title":"Pharmacological inhibition of the integrated stress response accelerates disease progression in an amyotrophic lateral sclerosis mouse model","volume":"181","author":"Marlin","year":"2023","journal-title":"Br. J. Pharmacol."}],"container-title":["Nucleic Acids Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/nar\/article-pdf\/52\/16\/9745\/59042444\/gkae655.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/nar\/article-pdf\/52\/16\/9745\/59042444\/gkae655.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T00:04:00Z","timestamp":1725840240000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/nar\/article\/52\/16\/9745\/7728025"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,6]]},"references-count":104,"journal-issue":{"issue":"16","published-online":{"date-parts":[[2024,8,6]]},"published-print":{"date-parts":[[2024,9,9]]}},"URL":"https:\/\/doi.org\/10.1093\/nar\/gkae655","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2023.11.07.566060","asserted-by":"object"}]},"ISSN":["0305-1048","1362-4962"],"issn-type":[{"value":"0305-1048","type":"print"},{"value":"1362-4962","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2024,9,9]]},"published":{"date-parts":[[2024,8,6]]}}}