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These repeats form stable RNA secondary structures that support aberrant translation in the absence of an AUG start codon (RAN translation), producing aggregate-prone peptides that accumulate within intranuclear neuronal inclusions and contribute to neurotoxicity. Here, we show that the most abundant RAN translation product, FMRpolyG, is markedly less toxic when generated from a construct with a non-repetitive alternating codon sequence in place of the CGG repeat. While exploring the mechanism of this differential toxicity, we observed a\u00a0+1 translational frameshift within the CGG repeat from the arginine to glycine reading frame. Frameshifts occurred within the first few translated repeats and were triggered predominantly by RNA sequence and structural features. Short chimeric R\/G peptides form aggregates distinct from those formed by either pure arginine or glycine, and these chimeras induce toxicity in cultured rodent neurons. Together, this work suggests that CGG repeats support translational frameshifting and that chimeric RAN translated peptides may contribute to CGG repeat-associated toxicity in FXTAS and related disorders.<\/jats:p>","DOI":"10.1093\/nar\/gkac626","type":"journal-article","created":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T15:39:41Z","timestamp":1659109181000},"page":"8674-8689","source":"Crossref","is-referenced-by-count":43,"title":["CGG repeats trigger translational frameshifts that generate aggregation-prone chimeric proteins"],"prefix":"10.1093","volume":"50","author":[{"given":"Shannon E","family":"Wright","sequence":"first","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, USA"},{"name":"Neuroscience Graduate Program, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caitlin M","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, USA"},{"name":"Neuroscience Graduate Program, University of Michigan , Ann Arbor , MI \u00a048109, USA"},{"name":"Department of Genetics, Stanford University School of Medicine , Stanford , CA \u00a084305, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy","family":"Monroe","sequence":"additional","affiliation":[{"name":"Department of Chemistry, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiazheng","family":"Xing","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Krans","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, USA"},{"name":"VA Ann Arbor Healthcare System , Ann Arbor , MI \u00a048105, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brittany\u00a0N","family":"Flores","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, USA"},{"name":"Cellular and Molecular Biology Graduate Program, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Venkatesha","family":"Barsur","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena I","family":"Ivanova","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, USA"},{"name":"Biophysics Program, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristin\u00a0S","family":"Koutmou","sequence":"additional","affiliation":[{"name":"Department of Chemistry, University of Michigan , Ann Arbor , MI \u00a048109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sami\u00a0J","family":"Barmada","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Michigan , Ann Arbor , MI \u00a048109, 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 \u00a048109, USA"},{"name":"VA Ann Arbor Healthcare System , Ann Arbor , MI \u00a048105, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,7,29]]},"reference":[{"key":"2022082523582743400_B1","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1038\/s41580-021-00382-6","article-title":"Molecular mechanisms underlying nucleotide repeat expansion disorders","volume":"22","author":"Malik","year":"2021","journal-title":"Nat. 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