{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T03:43:59Z","timestamp":1788493439455,"version":"build-2803163510"},"reference-count":39,"publisher":"Public Library of Science (PLoS)","issue":"7","license":[{"start":{"date-parts":[[2020,7,31]],"date-time":"2020-07-31T00:00:00Z","timestamp":1596153600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["APP1184166"],"award-info":[{"award-number":["APP1184166"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["APP1161803"],"award-info":[{"award-number":["APP1161803"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["APP1102059"],"award-info":[{"award-number":["APP1102059"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["APP1154352"],"award-info":[{"award-number":["APP1154352"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0233583","type":"journal-article","created":{"date-parts":[[2020,7,31]],"date-time":"2020-07-31T13:33:32Z","timestamp":1596202412000},"page":"e0233583","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":6,"title":["Nascent mutant Huntingtin exon 1 chains do not stall on ribosomes during translation but aggregates do recruit machinery involved in ribosome quality control and RNA"],"prefix":"10.1371","volume":"15","author":[{"given":"Angelique R.","family":"Ormsby","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5345-8360","authenticated-orcid":true,"given":"Dezerae","family":"Cox","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Daly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Priest","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Hinde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9965-2847","authenticated-orcid":true,"given":"Danny M.","family":"Hatters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2020,7,31]]},"reference":[{"issue":"6","key":"pone.0233583.ref001","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A Novel Gene Containing a Trinucleotide Repeat that is Expanded and Unstable on Huntington's Disease Chromosomes","volume":"72","author":"ME MacDonald","year":"1993","journal-title":"Cell"},{"issue":"8","key":"pone.0233583.ref002","doi-asserted-by":"crossref","first-page":"4604","DOI":"10.1073\/pnas.96.8.4604","article-title":"Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology","volume":"96","author":"E Scherzinger","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6","key":"pone.0233583.ref003","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1111\/bpa.12427","article-title":"RNA toxicity induced by expanded CAG repeats in Huntington's disease","volume":"26","author":"E. Mart\u00ed","year":"2016","journal-title":"Brain Pathol"},{"issue":"3","key":"pone.0233583.ref004","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/S0092-8674(00)80514-0","article-title":"Huntingtin-encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo","volume":"90","author":"E Scherzinger","year":"1997","journal-title":"Cell"},{"issue":"5334","key":"pone.0233583.ref005","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"M DiFiglia","year":"1997","journal-title":"Science"},{"issue":"3","key":"pone.0233583.ref006","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","article-title":"Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation","volume":"90","author":"SW Davies","year":"1997","journal-title":"Cell"},{"issue":"6","key":"pone.0233583.ref007","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1093\/hmg\/ddg075","article-title":"Transgenic rat model of Huntington's disease","volume":"12","author":"S von Horsten","year":"2003","journal-title":"Hum Mol Genet"},{"issue":"7197","key":"pone.0233583.ref008","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1038\/nature06975","article-title":"Towards a transgenic model of Huntington\/'s disease in a non-human primate","volume":"453","author":"S-H Yang","year":"2008","journal-title":"Nature"},{"issue":"5","key":"pone.0233583.ref009","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/j.celrep.2017.04.029","article-title":"Huntingtin Inclusions Trigger Cellular Quiescence, Deactivate Apoptosis, and Lead to Delayed Necrosis","volume":"19","author":"YM Ramdzan","year":"2017","journal-title":"Cell Rep"},{"issue":"12","key":"pone.0233583.ref010","doi-asserted-by":"crossref","first-page":"e15245","DOI":"10.1371\/journal.pone.0015245","article-title":"Formation and Toxicity of Soluble Polyglutamine Oligomers in Living Cells","volume":"5","author":"P Lajoie","year":"2011","journal-title":"PLoS ONE"},{"issue":"3","key":"pone.0233583.ref011","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1093\/hmg\/ddm311","article-title":"Soluble Polyglutamine Oligomers Formed Prior to Inclusion Body Formation are Cytotoxic","volume":"17","author":"T Takahashi","year":"2007","journal-title":"Hum Mol Genet"},{"key":"pone.0233583.ref012","doi-asserted-by":"crossref","first-page":"2753","DOI":"10.1038\/ncomms3753","article-title":"Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress","volume":"4","author":"J Leitman","year":"2013","journal-title":"Nat Commun"},{"issue":"19","key":"pone.0233583.ref013","doi-asserted-by":"crossref","first-page":"16017","DOI":"10.1074\/jbc.M111.252577","article-title":"Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein","volume":"287","author":"LG Nucifora","year":"2012","journal-title":"J Biol Chem"},{"issue":"22","key":"pone.0233583.ref014","doi-asserted-by":"crossref","first-page":"2611","DOI":"10.2174\/1568026611212220013","article-title":"Putting huntingtin \"aggregation\" in view with windows into the cellular milieu","volume":"12","author":"DM Hatters","year":"2012","journal-title":"Curr Top Med Chem"},{"issue":"4","key":"pone.0233583.ref015","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1016\/j.molcel.2018.04.007","article-title":"A Liquid to Solid Phase Transition Underlying Pathological Huntingtin Exon1 Aggregation","volume":"70","author":"TR Peskett","year":"2018","journal-title":"Mol Cell"},{"issue":"5","key":"pone.0233583.ref016","doi-asserted-by":"crossref","first-page":"e201900489","DOI":"10.26508\/lsa.201900489","article-title":"The huntingtin inclusion is a dynamic phase-separated compartment","volume":"2","author":"F Aktar","year":"2019","journal-title":"Life Sci Alliance"},{"issue":"4","key":"pone.0233583.ref017","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1074\/mcp.RA119.001888","article-title":"Arginine in C9ORF72 Dipolypeptides Mediates Promiscuous Proteome Binding and Multiple Modes of Toxicity","volume":"19","author":"M Radwan","year":"2020","journal-title":"Mol Cell Proteomics"},{"issue":"1","key":"pone.0233583.ref018","doi-asserted-by":"crossref","first-page":"2193","DOI":"10.1038\/s41598-017-02460-2","article-title":"Systematic comparison of 2A peptides for cloning multi-genes in a polycistronic vector","volume":"7","author":"Z Liu","year":"2017","journal-title":"Sci Rep"},{"issue":"5","key":"pone.0233583.ref019","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1038\/nmeth.1930","article-title":"Tracking protein aggregation and mislocalization in cells with flow cytometry","volume":"9","author":"YM Ramdzan","year":"2012","journal-title":"Nat Methods"},{"issue":"9","key":"pone.0233583.ref020","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1038\/nchembio.1308","article-title":"Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration","volume":"9","author":"AS Tsvetkov","year":"2013","journal-title":"Nat Chem Biol"},{"issue":"4","key":"pone.0233583.ref021","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1083\/jcb.201311103","article-title":"Protecting the proteome: Eukaryotic cotranslational quality control pathways","volume":"204","author":"J Lykke-Andersen","year":"2014","journal-title":"J Cell Biol"},{"issue":"4","key":"pone.0233583.ref022","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/j.molcel.2016.11.039","article-title":"Initiation of Quality Control during Poly(A) Translation Requires Site-Specific Ribosome Ubiquitination","volume":"65","author":"S Juszkiewicz","year":"2017","journal-title":"Mol Cell"},{"issue":"Pt 5","key":"pone.0233583.ref023","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1099\/0022-1317-82-5-1013","article-title":"Analysis of the aphthovirus 2A\/2B polyprotein 'cleavage' mechanism indicates not a proteolytic reaction, but a novel translational effect: a putative ribosomal 'skip'","volume":"82","author":"ML Donnelly","year":"2001","journal-title":"J Gen Virol"},{"issue":"28","key":"pone.0233583.ref024","doi-asserted-by":"crossref","first-page":"21807","DOI":"10.1074\/jbc.M109.084434","article-title":"Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool","volume":"285","author":"MA Olshina","year":"2010","journal-title":"J Biol Chem"},{"issue":"41","key":"pone.0233583.ref025","doi-asserted-by":"crossref","first-page":"15779","DOI":"10.1073\/pnas.0808480105","article-title":"Exploring RNA transcription and turnover in vivo by using click chemistry","volume":"105","author":"CY Jao","year":"2008","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"1","key":"pone.0233583.ref026","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1038\/nsmb.3147","article-title":"Ribosome-associated protein quality control","volume":"23","author":"O Brandman","year":"2016","journal-title":"Nat Struct Mol Biol"},{"issue":"1","key":"pone.0233583.ref027","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1146\/annurev-cellbio-111315-125249","article-title":"Ribosomal Stalling During Translation: Providing Substrates for Ribosome-Associated Protein Quality Control","volume":"33","author":"CAP Joazeiro","year":"2017","journal-title":"Annual Review of Cell and Developmental Biology"},{"issue":"12","key":"pone.0233583.ref028","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1038\/embor.2010.169","article-title":"Receptor for activated C kinase 1 stimulates nascent polypeptide-dependent translation arrest","volume":"11","author":"K Kuroha","year":"2010","journal-title":"EMBO Rep"},{"issue":"13","key":"pone.0233583.ref029","doi-asserted-by":"crossref","first-page":"2510","DOI":"10.1128\/MCB.01362-12","article-title":"Degradation of newly synthesized polypeptides by ribosome-associated RACK1\/c-Jun N-terminal kinase\/eukaryotic elongation factor 1A2 complex","volume":"33","author":"V Gandin","year":"2013","journal-title":"Mol Cell Biol"},{"issue":"26","key":"pone.0233583.ref030","doi-asserted-by":"crossref","first-page":"21599","DOI":"10.1074\/jbc.M112.359307","article-title":"Proteomic analysis of wild-type and mutant huntingtin-associated proteins in mouse brains identifies unique interactions and involvement in protein synthesis","volume":"287","author":"BP Culver","year":"2012","journal-title":"J Biol Chem"},{"issue":"4","key":"pone.0233583.ref031","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1016\/j.molcel.2016.12.026","article-title":"ZNF598 and RACK1 Regulate Mammalian Ribosome-Associated Quality Control Function by Mediating Regulatory 40S Ribosomal Ubiquitylation","volume":"65","author":"E Sundaramoorthy","year":"2017","journal-title":"Mol Cell"},{"issue":"6220","key":"pone.0233583.ref032","doi-asserted-by":"crossref","first-page":"1260419","DOI":"10.1126\/science.1260419","article-title":"Proteomics. Tissue-based map of the human proteome","volume":"347","author":"M Uhlen","year":"2015","journal-title":"Science"},{"key":"pone.0233583.ref033","first-page":"629667","article-title":"Global ribosome profiling reveals that mutant huntingtin stalls ribosomes and represses protein synthesis independent of fragile X mental retardation protein","author":"M Eshraghi","year":"2019","journal-title":"bioRxiv"},{"issue":"6","key":"pone.0233583.ref034","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.1500154","article-title":"Translational control by lysine-encoding A-rich sequences","volume":"1","author":"L Arthur","year":"2015","journal-title":"Sci Adv"},{"issue":"12","key":"pone.0233583.ref035","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1038\/s41594-019-0331-x","article-title":"Mechanism of ribosome stalling during translation of a poly(A) tail","volume":"26","author":"V Chandrasekaran","year":"2019","journal-title":"Nat Struct Mol Biol"},{"issue":"9","key":"pone.0233583.ref036","doi-asserted-by":"crossref","first-page":"2100","DOI":"10.1016\/j.celrep.2017.08.018","article-title":"Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function","volume":"20","author":"FF Morelli","year":"2017","journal-title":"Cell Rep"},{"issue":"4","key":"pone.0233583.ref037","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1038\/s41594-020-0399-3","article-title":"Hsp27 chaperones FUS phase separation under the modulation of stress-induced phosphorylation","volume":"27","author":"Z Liu","year":"2020","journal-title":"Nat Struct Mol Biol"},{"issue":"6357","key":"pone.0233583.ref038","doi-asserted-by":"crossref","DOI":"10.1126\/science.aaf4382","article-title":"Liquid phase condensation in cell physiology and disease","volume":"357","author":"Y Shin","year":"2017","journal-title":"Science"},{"issue":"12","key":"pone.0233583.ref039","doi-asserted-by":"crossref","first-page":"3078","DOI":"10.1016\/j.bpj.2010.02.058","article-title":"A two-step path to inclusion formation of huntingtin peptides revealed by number and brightness analysis","volume":"98","author":"G Ossato","year":"2010","journal-title":"Biophys J"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0233583","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,7,31]],"date-time":"2020-07-31T13:34:10Z","timestamp":1596202450000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0233583"}},"subtitle":[],"editor":[{"given":"David R.","family":"Borchelt","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2020,7,31]]},"references-count":39,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2020,7,31]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0233583","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2020.05.13.094060","asserted-by":"object"}]},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,7,31]]}}}