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These GC-rich repeats form RNA secondary structures that support repeat-associated non-AUG (RAN) translation of toxic proteins that contribute to disease pathogenesis. Here we assessed whether these same repeats might trigger stalling and interfere with translational elongation. We find that depletion of ribosome-associated quality control (RQC) factors NEMF, LTN1\u00a0and ANKZF1 markedly boost RAN translation product accumulation from both G4C2 and CGG repeats while overexpression of these factors reduces RAN production in both reporter assays and C9ALS\/FTD patient iPSC-derived neurons. We also detected partially made products from both G4C2 and CGG repeats whose abundance increased with RQC factor depletion. Repeat RNA sequence, rather than amino acid content, is central to the impact of RQC factor depletion on RAN translation\u2014suggesting a role for RNA secondary structure in these processes. Together, these findings suggest that ribosomal stalling and RQC pathway activation during RAN translation inhibits the generation of toxic RAN products. We propose augmenting RQC activity as a therapeutic strategy in GC-rich repeat expansion disorders.<\/jats:p>","DOI":"10.1093\/nar\/gkae137","type":"journal-article","created":{"date-parts":[[2024,2,19]],"date-time":"2024-02-19T17:00:46Z","timestamp":1708362046000},"page":"5928-5949","source":"Crossref","is-referenced-by-count":23,"title":["Ribosomal quality control factors inhibit repeat-associated non-AUG translation from GC-rich repeats"],"prefix":"10.1093","volume":"52","author":[{"given":"Yi-Ju","family":"Tseng","sequence":"first","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"},{"name":"Cellular and Molecular Biology Graduate Program, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Krans","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"},{"name":"Ann Arbor Veterans Administration Healthcare , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Indranil","family":"Malik","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"},{"name":"Department of Biotechnology, Indian Institute of Technology Hyderabad , Kandi , Sangareddy, 502284 \u00a0 Telangana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiexiong","family":"Deng","sequence":"additional","affiliation":[{"name":"Department of Molecular, Cellular and Developmental Biology, Howard Hughes Medical Institute, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evrim","family":"Yildirim","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sinem","family":"Ovunc","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth\u00a0M H","family":"Tank","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Jansen-West","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic , Jacksonville , FL\u00a0 32224 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ross","family":"Kaufhold","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"},{"name":"Medical Scientist Training Program, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas\u00a0B","family":"Gomez","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"},{"name":"Medical Scientist Training Program, University of Michigan , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger","family":"Sher","sequence":"additional","affiliation":[{"name":"Department of Neurobiology and Behavior & Center for Nervous System Disorders, Stony Brook University , Stony Brook , NY\u00a0 11794 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonard","family":"Petrucelli","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic , Jacksonville , FL\u00a0 32224 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sami\u00a0J","family":"Barmada","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI\u00a0 48109 , 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 , Ann Arbor , MI\u00a0 48109 , USA"},{"name":"Ann Arbor Veterans Administration Healthcare , Ann Arbor , MI\u00a0 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,2,27]]},"reference":[{"key":"2024060923564020500_B1","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1016\/j.neuron.2013.02.022","article-title":"The unstable repeats-three evolving faces of neurological disease","volume":"77","author":"Nelson","year":"2013","journal-title":"Neuron"},{"key":"2024060923564020500_B2","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1038\/7710","article-title":"An 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Rev. Mol. Cell Biol."},{"key":"2024060923564020500_B7","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.brainres.2016.04.003","article-title":"RAN translation\u2014What makes it run?","volume":"1647","author":"Green","year":"2016","journal-title":"Brain Res."},{"key":"2024060923564020500_B8","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1073\/pnas.1013343108","article-title":"Non-ATG-initiated translation directed by microsatellite expansions","volume":"108","author":"Zu","year":"2011","journal-title":"Proc. Natl. Acad. Sci."},{"key":"2024060923564020500_B9","doi-asserted-by":"crossref","first-page":"R45","DOI":"10.1093\/hmg\/ddt371","article-title":"Repeat-associated non-ATG (RAN) translation in neurological disease","volume":"22","author":"Cleary","year":"2013","journal-title":"Hum. Mol. 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Dev."},{"key":"2024060923564020500_B12","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/j.neuron.2017.01.001","article-title":"Microsatellite expansion diseases: repeat toxicity found in translation","volume":"93","author":"Gao","year":"2017","journal-title":"Neuron"},{"key":"2024060923564020500_B13","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1016\/j.neuron.2015.10.038","article-title":"RAN translation in Huntington Disease","volume":"88","author":"Ba\u00f1ez-Coronel","year":"2015","journal-title":"Neuron"},{"key":"2024060923564020500_B14","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1007\/s00401-013-1189-3","article-title":"Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins","volume":"126","author":"Mori","year":"2013","journal-title":"Acta Neuropathol."},{"key":"2024060923564020500_B15","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":"2024060923564020500_B16","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":"Xinmei","year":"2014","journal-title":"Neuron"},{"key":"2024060923564020500_B17","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.neuron.2016.12.016","article-title":"Translation of expanded CGG repeats into FMRpolyG is pathogenic and may contribute to fragile X tremor ataxia syndrome","volume":"93","author":"Sellier","year":"2017","journal-title":"Neuron"},{"key":"2024060923564020500_B18","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1093\/humrep\/dev280","article-title":"Presence of inclusions positive for polyglycine containing protein, FMRpolyG, indicates that repeat-associated non-AUG translation plays a role in fragile X-associated primary ovarian insufficiency","volume":"31","author":"Buijsen","year":"2016","journal-title":"Hum. Reprod."},{"key":"2024060923564020500_B19","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1186\/s40478-014-0162-2","article-title":"FMRpolyG-positive inclusions in CNS and non-CNS organs of a fragile X premutation carrier with fragile X-associated tremor\/ataxia syndrome","volume":"2","author":"Buijsen","year":"2014","journal-title":"Acta Neuropathol. 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Ophthalmol. Vis. Sci."},{"key":"2024060923564020500_B24","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1016\/j.neuron.2017.08.039","article-title":"RAN translation regulated by muscleblind proteins in myotonic dystrophy type 2","volume":"95","author":"Zu","year":"2017","journal-title":"Neuron"},{"key":"2024060923564020500_B25","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":"2024060923564020500_B26","doi-asserted-by":"crossref","first-page":"e2118124119","DOI":"10.1073\/pnas.2118124119","article-title":"Identification of PSMB5 as a genetic modifier of fragile X\u2013associated tremor\/ataxia syndrome","volume":"119","author":"Kong","year":"2022","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2024060923564020500_B27","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":"2024060923564020500_B28","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":"2024060923564020500_B29","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1038\/s41593-019-0455-7","article-title":"RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats","volume":"22","author":"Yamada","year":"2019","journal-title":"Nat. Neurosci."},{"key":"2024060923564020500_B30","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1093\/jb\/mvad012","article-title":"Repeat-associated non-AUG translation in neuromuscular diseases: mechanisms and therapeutic insights","volume":"173","author":"Fujino","year":"2023","journal-title":"J. Bioch."},{"key":"2024060923564020500_B31","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":"2024060923564020500_B32","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1002\/humu.22244","article-title":"A Pan-European study of the C9orf72 repeat associated with FTLD: geographic prevalence, genomic instability, and intermediate repeats","volume":"34","author":"van\u00a0der\u00a0Zee","year":"2013","journal-title":"Hum. 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Opin. Neurol."},{"key":"2024060923564020500_B38","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.chembiol.2018.10.018","article-title":"The hairpin form of r (G 4 C 2) exp in c9ALS \/FTD is repeat-associated non-ATG translated and a target for bioactive small molecules article The hairpin form of r (G 4 C 2) exp in c9ALS \/ FTD is repeat-associated non-ATG translated and a target for B","volume":"26","author":"Wang","year":"2019","journal-title":"Cell Chem Biol"},{"key":"2024060923564020500_B39","doi-asserted-by":"crossref","first-page":"eabd9440","DOI":"10.1126\/sciadv.abd9440","article-title":"CGG repeat RNA G-quadruplexes interact with FMRpolyG to cause neuronal dysfunction in fragile X-related tremor\/ataxia syndrome","volume":"7","author":"Asamitsu","year":"2021","journal-title":"Sci. Adv."},{"key":"2024060923564020500_B40","doi-asserted-by":"crossref","first-page":"e17820","DOI":"10.7554\/eLife.17820","article-title":"The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains","volume":"5","author":"Conlon","year":"2016","journal-title":"eLife"},{"key":"2024060923564020500_B41","doi-asserted-by":"crossref","first-page":"4653","DOI":"10.1074\/jbc.C113.502336","article-title":"TMPyP4 porphyrin distorts RNA G-quadruplex structures of the disease-associated r(GGGGCC)n repeat of the C9orf72 gene and blocks interaction of RNAbinding proteins","volume":"289","author":"Zamiri","year":"2014","journal-title":"J. Biol. 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