{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T08:43:46Z","timestamp":1785833026146,"version":"3.56.0"},"reference-count":64,"publisher":"Elsevier BV","issue":"11","license":[{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T00:00:00Z","timestamp":1758585600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009108","name":"Shandong University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009108","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007046","name":"Wuhan University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007046","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["32270825"],"award-info":[{"award-number":["32270825"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005492","name":"Stanford University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005492","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100010029","name":"Taishan Scholar Foundation of Shandong Province","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100010029","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007129","name":"Natural Science Foundation of Shandong Province","doi-asserted-by":"publisher","award":["2023HWYQ-013"],"award-info":[{"award-number":["2023HWYQ-013"]}],"id":[{"id":"10.13039\/501100007129","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007129","name":"Natural Science Foundation of Shandong Province","doi-asserted-by":"publisher","award":["tsqn202306046"],"award-info":[{"award-number":["tsqn202306046"]}],"id":[{"id":"10.13039\/501100007129","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["jbc.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Biological Chemistry"],"published-print":{"date-parts":[[2025,11]]},"DOI":"10.1016\/j.jbc.2025.110764","type":"journal-article","created":{"date-parts":[[2025,9,24]],"date-time":"2025-09-24T17:28:08Z","timestamp":1758734888000},"page":"110764","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":4,"title":["IRE1\/Xbp1 promotes the clearance of poly(GR) dipeptide repeats in amyotrophic lateral sclerosis"],"prefix":"10.1016","volume":"301","author":[{"given":"Yu","family":"Li","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dongyue","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuangxi","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jbc.2025.110764_bib1","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.jbc.2025.110764_bib2","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.jbc.2025.110764_bib3","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1016\/j.neuron.2020.08.022","article-title":"ALS genetics: gains, losses, and implications for future therapies","volume":"108","author":"Kim","year":"2020","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib4","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.jbc.2025.110764_bib5","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":"10.1016\/j.jbc.2025.110764_bib6","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/j.neuron.2013.02.004","article-title":"Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD\/ALS","volume":"77","author":"Ash","year":"2013","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib7","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1016\/j.cell.2017.10.042","article-title":"Rethinking unconventional translation in neurodegeneration","volume":"171","author":"Gao","year":"2017","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib8","doi-asserted-by":"crossref","first-page":"a024224","DOI":"10.1101\/cshperspect.a024224","article-title":"Disease mechanisms of C9ORF72 repeat expansions","volume":"8","author":"Gendron","year":"2018","journal-title":"Cold Spring Harb. Perspect. Med."},{"key":"10.1016\/j.jbc.2025.110764_bib9","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1016\/j.cell.2022.12.032","article-title":"Hallmarks of neurodegenerative diseases","volume":"186","author":"Wilson","year":"2023","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib10","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":"Jovicic","year":"2015","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.jbc.2025.110764_bib11","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1097\/WCO.0b013e32835a3efb","article-title":"How do C9ORF72 repeat expansions cause amyotrophic lateral sclerosis and frontotemporal dementia: can we learn from other noncoding repeat expansion disorders?","volume":"25","author":"van Blitterswijk","year":"2012","journal-title":"Curr. Opin. Neurol."},{"key":"10.1016\/j.jbc.2025.110764_bib12","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.jbc.2025.110764_bib13","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1016\/j.cell.2011.10.011","article-title":"The seeds of neurodegeneration: prion-like spreading in ALS","volume":"147","author":"Polymenidou","year":"2011","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib14","doi-asserted-by":"crossref","first-page":"3434","DOI":"10.1016\/j.neuron.2024.07.025","article-title":"Disruption of nuclear speckle integrity dysregulates RNA splicing in C9ORF72-FTD\/ALS","volume":"112","author":"Wu","year":"2024","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib15","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1186\/s40478-018-0564-7","article-title":"Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease","volume":"6","author":"Sakae","year":"2018","journal-title":"Acta Neuropathol. Commun."},{"key":"10.1016\/j.jbc.2025.110764_bib16","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1007\/s00401-019-02050-8","article-title":"Dipeptide repeat (DPR) pathology in the skeletal muscle of ALS patients with C9ORF72 repeat expansion","volume":"138","author":"Cykowski","year":"2019","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.jbc.2025.110764_bib17","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.jbc.2025.110764_bib18","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1038\/s41593-019-0397-0","article-title":"C9ORF72-ALS\/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo","volume":"22","author":"Choi","year":"2019","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.jbc.2025.110764_bib19","doi-asserted-by":"crossref","first-page":"25104","DOI":"10.1073\/pnas.2005506117","article-title":"Quality-control mechanisms targeting translationally stalled and C-terminally extended poly(GR) associated with ALS\/FTD","volume":"117","author":"Li","year":"2020","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2025.110764_bib20","article-title":"The mTORC2\/AKT\/VCP axis is associated with quality control of the stalled translation of poly(GR) dipeptide repeats in C9-ALS\/FTD","volume":"299","author":"Li","year":"2023","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2025.110764_bib21","doi-asserted-by":"crossref","first-page":"1042","DOI":"10.1016\/j.cell.2012.10.044","article-title":"A ribosome-bound quality control complex triggers degradation of nascent peptides and signals translation stress","volume":"151","author":"Brandman","year":"2012","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib22","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1016\/j.cell.2020.06.006","article-title":"Ribosome collisions trigger general stress responses to regulate cell fate","volume":"182","author":"Wu","year":"2020","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib23","doi-asserted-by":"crossref","DOI":"10.1126\/scisignal.adl1030","article-title":"Poly-GR repeats associated with ALS\/FTD gene C9ORF72 impair translation elongation and induce a ribotoxic stress response in neurons","volume":"17","author":"Dong","year":"2024","journal-title":"Sci. Signal."},{"key":"10.1016\/j.jbc.2025.110764_bib24","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1007\/s00294-017-0708-5","article-title":"The ribosome-bound quality control complex: from aberrant peptide clearance to proteostasis maintenance","volume":"63","author":"Defenouillere","year":"2017","journal-title":"Curr. Genet."},{"key":"10.1016\/j.jbc.2025.110764_bib25","doi-asserted-by":"crossref","first-page":"1136","DOI":"10.1038\/s41591-018-0071-1","article-title":"Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis","volume":"24","author":"Zhang","year":"2018","journal-title":"Nat. Med."},{"key":"10.1016\/j.jbc.2025.110764_bib26","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a013169","article-title":"Endoplasmic reticulum stress sensing in the unfolded protein response","volume":"5","author":"Gardner","year":"2013","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib27","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.molcel.2017.06.017","article-title":"The unfolded protein response and cell fate control","volume":"69","author":"Hetz","year":"2018","journal-title":"Mol. Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib28","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1038\/nrm1052","article-title":"Quality control in the endoplasmic reticulum","volume":"4","author":"Ellgaard","year":"2003","journal-title":"Nat. Rev. Mol. Cell Biol"},{"key":"10.1016\/j.jbc.2025.110764_bib29","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1038\/nrm2199","article-title":"Signal integration in the endoplasmic reticulum unfolded protein response","volume":"8","author":"Ron","year":"2007","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib30","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/j.molcel.2009.08.021","article-title":"Fine-tuning of the unfolded protein response: assembling the IRE1alpha interactome","volume":"35","author":"Hetz","year":"2009","journal-title":"Mol. Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib31","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1038\/nrm3270","article-title":"The unfolded protein response: controlling cell fate decisions under ER stress and beyond","volume":"13","author":"Hetz","year":"2012","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib32","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1016\/S0092-8674(01)00611-0","article-title":"XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor","volume":"107","author":"Yoshida","year":"2001","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib33","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1038\/sj.emboj.7601477","article-title":"Unfolded protein response in a Drosophila model for retinal degeneration","volume":"26","author":"Ryoo","year":"2007","journal-title":"EMBO J."},{"key":"10.1016\/j.jbc.2025.110764_bib34","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1091\/mbc.e11-08-0663","article-title":"IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin","volume":"23","author":"Chiang","year":"2012","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib35","doi-asserted-by":"crossref","first-page":"2018","DOI":"10.1016\/j.cmet.2022.10.010","article-title":"Cancer cell-intrinsic XBP1 drives immunosuppressive reprogramming of intratumoral myeloid cells by promoting cholesterol production","volume":"34","author":"Yang","year":"2022","journal-title":"Cell Metab."},{"key":"10.1016\/j.jbc.2025.110764_bib36","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2023.113303","article-title":"Differential Ire1 determines Loser cell fate in tumor-suppressive cell competition","volume":"42","author":"Zheng","year":"2023","journal-title":"Cell Rep."},{"key":"10.1016\/j.jbc.2025.110764_bib37","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1111\/febs.14332","article-title":"Emerging roles of ER stress in the etiology and pathogenesis of Alzheimer's disease","volume":"285","author":"Gerakis","year":"2018","journal-title":"FEBS J."},{"key":"10.1016\/j.jbc.2025.110764_bib38","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1038\/ni.3709","article-title":"The metabolic ER stress sensor IRE1alpha suppresses alternative activation of macrophages and impairs energy expenditure in obesity","volume":"18","author":"Shan","year":"2017","journal-title":"Nat. Immunol."},{"key":"10.1016\/j.jbc.2025.110764_bib39","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1242\/dev.118.2.401","article-title":"Targeted gene expression as a means of altering cell fates and generating dominant phenotypes","volume":"118","author":"Brand","year":"1993","journal-title":"Development"},{"key":"10.1016\/j.jbc.2025.110764_bib40","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2020.107989","article-title":"Altered MICOS morphology and mitochondrial ion homeostasis contribute to Poly(GR) toxicity associated with C9-ALS\/FTD","volume":"32","author":"Li","year":"2020","journal-title":"Cell Rep."},{"key":"10.1016\/j.jbc.2025.110764_bib41","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.brainres.2014.09.064","article-title":"A fruitful endeavor: modeling ALS in the fruit fly","volume":"1607","author":"Casci","year":"2015","journal-title":"Brain Res."},{"key":"10.1016\/j.jbc.2025.110764_bib42","doi-asserted-by":"crossref","first-page":"1052","DOI":"10.1038\/s41589-020-0584-z","article-title":"Pharmacologic IRE1\/XBP1s activation confers targeted ER proteostasis reprogramming","volume":"16","author":"Grandjean","year":"2020","journal-title":"Nat. Chem. Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib43","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1038\/s41467-022-28271-2","article-title":"Pharmacologic IRE1\/XBP1s activation promotes systemic adaptive remodeling in obesity","volume":"13","author":"Madhavan","year":"2022","journal-title":"Nat. Commun."},{"key":"10.1016\/j.jbc.2025.110764_bib44","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1038\/s41580-019-0133-3","article-title":"The Hsp70 chaperone network","volume":"20","author":"Rosenzweig","year":"2019","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib45","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.1903936116","article-title":"The Hsp70 chaperone is a major player in stress-induced transposable element activation","volume":"116","author":"Cappucci","year":"2019","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2025.110764_bib46","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2202133119","article-title":"Bip-Yorkie interaction determines oncogenic and tumor-suppressive roles of Ire1\/Xbp1s activation","volume":"119","author":"Yang","year":"2022","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2025.110764_bib47","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1038\/nsmb.3147","article-title":"Ribosome-associated protein quality control","volume":"23","author":"Brandman","year":"2016","journal-title":"Nat. Struct. Mol. Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib48","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1126\/science.1259724","article-title":"Protein synthesis. Rqc2p and 60S ribosomal subunits mediate mRNA-independent elongation of nascent chains","volume":"347","author":"Shen","year":"2015","journal-title":"Science"},{"key":"10.1016\/j.jbc.2025.110764_bib49","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1038\/s41594-019-0324-9","article-title":"UPR proteins IRE1 and PERK switch BiP from chaperone to ER stress sensor","volume":"26","author":"Kopp","year":"2019","journal-title":"Nat. Struct. Mol. Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib61","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1016\/j.ymthe.2025.01.004","article-title":"Artificial enforcement of the unfolded protein response reduces disease features in multiple preclinical models of ALS\/FTD","volume":"33","author":"Valenzuela","year":"2025","journal-title":"Mol. Ther."},{"key":"10.1016\/j.jbc.2025.110764_bib62","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2025.115459","article-title":"Differential neuronal vulnerability to C9orf72 repeat expansion driven by Xbp1-induced endoplasmic reticulum-associated degradation","volume":"44","author":"Shen","year":"2025","journal-title":"Cell Rep."},{"key":"10.1016\/j.jbc.2025.110764_bib50","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1016\/j.neuron.2016.04.005","article-title":"C9orf72 BAC mouse model with motor deficits and neurodegenerative features of ALS\/FTD","volume":"90","author":"Liu","year":"2016","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib51","doi-asserted-by":"crossref","DOI":"10.1016\/j.expneurol.2024.114768","article-title":"DDX3X overexpression decreases dipeptide repeat proteins in a mouse model of C9ORF72-ALS\/FTD","volume":"376","author":"Fu","year":"2024","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.jbc.2025.110764_bib52","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 vitro and in vivo neuronal death","volume":"84","author":"Wen","year":"2014","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib53","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.1016\/j.neuron.2025.02.005","article-title":"PTPsigma-mediated PI3P regulation modulates neurodegeneration in C9ORF72-ALS\/FTD","volume":"113","author":"Zhang","year":"2025","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib54","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/s00401-017-1694-x","article-title":"IRE1 signaling exacerbates Alzheimer's disease pathogenesis","volume":"134","author":"Duran-Aniotz","year":"2017","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.jbc.2025.110764_bib55","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":"10.1016\/j.jbc.2025.110764_bib56","doi-asserted-by":"crossref","first-page":"13173","DOI":"10.1523\/JNEUROSCI.4057-07.2007","article-title":"Exogenous delivery of heat shock protein 70 increases lifespan in a mouse model of amyotrophic lateral sclerosis","volume":"27","author":"Gifondorwa","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1016\/j.jbc.2025.110764_bib57","doi-asserted-by":"crossref","first-page":"1949","DOI":"10.1016\/j.neuron.2021.04.023","article-title":"UBQLN2-HSP70 axis reduces poly-gly-ala aggregates and alleviates behavioral defects in the C9ORF72 animal model","volume":"109","author":"Zhang","year":"2021","journal-title":"Neuron"},{"key":"10.1016\/j.jbc.2025.110764_bib58","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/j.molcel.2018.08.037","article-title":"ZNF598 is a quality control sensor of collided ribosomes","volume":"72","author":"Juszkiewicz","year":"2018","journal-title":"Mol. Cell"},{"key":"10.1016\/j.jbc.2025.110764_bib59","doi-asserted-by":"crossref","first-page":"11294","DOI":"10.1093\/nar\/gkab834","article-title":"ZNF598 co-translationally titrates poly(GR) protein implicated in the pathogenesis of C9ORF72-associated ALS\/FTD","volume":"49","author":"Park","year":"2021","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.jbc.2025.110764_bib60","doi-asserted-by":"crossref","first-page":"3186","DOI":"10.1523\/JNEUROSCI.1799-22.2023","article-title":"c-Jun N-Terminal kinase promotes stress Granule assembly and neurodegeneration in C9orf72-Mediated ALS and FTD","volume":"43","author":"Sahana","year":"2023","journal-title":"J. Neurosci."},{"key":"10.1016\/j.jbc.2025.110764_bib63","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.ydbio.2023.12.007","article-title":"The IRE1\/Xbp1 axis restores ER and tissue homeostasis perturbed by excess Notch in Drosophila","volume":"507","author":"Li","year":"2024","journal-title":"Dev. Biol."},{"key":"10.1016\/j.jbc.2025.110764_bib64","article-title":"RACK1 and IRE1 participate in the translational quality control of amyloid precursor protein in Drosophila models of Alzheimer's disease","volume":"300","author":"Li","year":"2024","journal-title":"J. Biol. Chem."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S002192582502616X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S002192582502616X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T03:46:14Z","timestamp":1773200774000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S002192582502616X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11]]},"references-count":64,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2025,11]]}},"alternative-id":["S002192582502616X"],"URL":"https:\/\/doi.org\/10.1016\/j.jbc.2025.110764","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2025,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"IRE1\/Xbp1 promotes the clearance of poly(GR) dipeptide repeats in amyotrophic lateral sclerosis","name":"articletitle","label":"Article Title"},{"value":"Journal of Biological Chemistry","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2025.110764","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Authors. Published by Elsevier Inc on behalf of American Society for Biochemistry and Molecular Biology\u00e9","name":"copyright","label":"Copyright"}],"article-number":"110764"}}