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Their expansion underlies the pathogenesis of multiple neurological disorders, including Huntington's disease, amyotrophic lateral sclerosis, and frontotemporal dementia, fragile X\u2010associated tremor\/ataxia syndrome, and myotonic dystrophies, known as repeat expansion disorders (REDs). Several molecular pathomechanisms associated with toxic RNA containing expanded repeats (RNA<jats:sup>exp<\/jats:sup>) are shared among REDs and contribute to disease progression, however, detailed mechanistic insight into those processes is limited. To deepen our understanding of the interplay between toxic RNA<jats:sup>exp<\/jats:sup> molecules and multiple protein partners, in this review, we discuss the roles of selected RNA\u2010binding proteins (RBPs) that interact with RNA<jats:sup>exp<\/jats:sup> and thus act as \u201cpartners in crime\u201d in the progression of REDs. We gather current findings concerning RBPs involved at different stages of the RNA<jats:sup>exp<\/jats:sup> life cycle, such as transcription, splicing, transport, and AUG\u2010independent translation of expanded repeats. We argue that the activity of selected RBPs can be unique or common among REDs depending on the expanded repeat type. We also present proteins that are functionally depleted due to sequestration on RNA<jats:sup>exp<\/jats:sup> within nuclear foci and those which participate in RNA<jats:sup>exp<\/jats:sup>\u2010dependent innate immunity activation. Moreover, we discuss the utility of selected RBPs as targets in the development of therapeutic strategies.<\/jats:p><jats:p>This article is categorized under:<jats:list list-type=\"simple\">\n<jats:list-item><jats:p>RNA Interactions with Proteins and Other Molecules &gt; Protein\u2010RNA Interactions: Functional Implications<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>RNA in Disease and Development &gt; RNA in Disease<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p>","DOI":"10.1002\/wrna.1709","type":"journal-article","created":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T07:52:12Z","timestamp":1646121132000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Partners in crime: Proteins implicated in <scp>RNA<\/scp> repeat expansion diseases"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3710-5722","authenticated-orcid":false,"given":"Anna","family":"Baud","sequence":"first","affiliation":[{"name":"Department of Gene Expression Institute of Molecular Biology and Biotechnology, Adam Mickiewicz University  Poznan Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magdalena","family":"Derbis","sequence":"additional","affiliation":[{"name":"Department of Gene Expression Institute of Molecular Biology and Biotechnology, Adam Mickiewicz University  Poznan Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2801-9956","authenticated-orcid":false,"given":"Katarzyna","family":"Tutak","sequence":"additional","affiliation":[{"name":"Department of Gene Expression Institute of Molecular Biology and Biotechnology, Adam Mickiewicz University  Poznan Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8352-9812","authenticated-orcid":false,"given":"Krzysztof","family":"Sobczak","sequence":"additional","affiliation":[{"name":"Department of Gene Expression Institute of Molecular Biology and Biotechnology, Adam Mickiewicz University  Poznan 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