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Different pathogenic mechanisms may underlie these non\u2010coding repeat expansion disorders. While gain\u2010of\u2010function mechanisms, such as toxicity associated with expression of repeat RNA or toxicity associated with repeat\u2010associated non\u2010ATG (RAN) products, are most frequently connected with these disorders, loss\u2010of\u2010function mechanisms have also been implicated. We review the different pathways that have been linked to non\u2010coding repeat expansion disorders such as C9ORF72\u2010linked ALS\/frontotemporal dementia (FTD), myotonic dystrophy, fragile X tremor\/ataxia syndrome (FXTAS), SCA, and Huntington's disease\u2010like 2. We discuss modes of RNA toxicity focusing on the identity and the interacting partners of the toxic RNA species. Using the C9ORF72 ALS\/FTD paradigm, we further explore the efforts and different methods used to disentangle RNA vs. RAN toxicity. Overall, we conclude that there is ample evidence for a role of RNA toxicity in non\u2010coding repeat expansion diseases.<\/jats:p>","DOI":"10.15252\/embj.2018101112","type":"journal-article","created":{"date-parts":[[2019,11,13]],"date-time":"2019-11-13T04:21:06Z","timestamp":1573618866000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":154,"title":["RNA toxicity in non\u2010coding repeat expansion disorders"],"prefix":"10.1038","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8098-880X","authenticated-orcid":false,"given":"Bart","family":"Swinnen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wim","family":"Robberecht","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0104-4067","authenticated-orcid":false,"given":"Ludo","family":"Van Den Bosch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,11,13]]},"reference":[{"key":"BFEMBJ2018101112_CR501","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1007\/s00439-004-1086-x","volume":"114","author":"EG Allen","year":"2004","unstructured":"Allen EG, He W, Yadav\u2010Shah M, Sherman SL (2004) A study of the distributional characteristics of FMR1 transcript levels in 238 individuals. 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