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CGG repeat RNAs sequester RNA\u2010binding proteins (RBPs) into nuclear foci and undergo repeat\u2010associated non\u2010AUG (RAN) translation into toxic peptides. To identify proteins involved in these processes, we employed a CGG repeat RNA\u2010tagging system to capture repeat\u2010associated RBPs by mass spectrometry in mammalian cells. We identified several SR (serine\/arginine\u2010rich) proteins that interact selectively with CGG repeats basally and under cellular stress. These proteins modify toxicity in a\n                    <jats:italic>Drosophila<\/jats:italic>\n                    model of FXTAS. Pharmacologic inhibition of serine\/arginine protein kinases (SRPKs), which alter SRSF protein phosphorylation, localization, and activity, directly inhibits RAN translation of CGG and GGGGCC repeats (associated with C9orf72 ALS\/FTD) and triggers repeat RNA retention in the nucleus. Lowering SRPK expression suppressed toxicity in both FXTAS and C9orf72 ALS\/FTD model flies, and SRPK inhibitors suppressed CGG repeat toxicity in rodent neurons. Together, these findings demonstrate roles for CGG repeat RNA binding proteins in RAN translation and repeat toxicity and support further evaluation of SRPK inhibitors in modulating RAN translation associated with repeat expansion disorders.\n                  <\/jats:p>","DOI":"10.15252\/emmm.202114163","type":"journal-article","created":{"date-parts":[[2021,9,20]],"date-time":"2021-09-20T09:38:28Z","timestamp":1632130708000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["SRSF protein kinase 1 modulates RAN translation and suppresses CGG repeat toxicity"],"prefix":"10.1038","volume":"13","author":[{"given":"Indranil","family":"Malik","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi\u2010Ju","family":"Tseng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon E","family":"Wright","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prithika","family":"Ramaiyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katelyn M","family":"Green","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4781-6376","authenticated-orcid":false,"given":"Peter K","family":"Todd","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,9,20]]},"reference":[{"key":"BFEMMM202114163_CR1","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1016\/j.ccr.2011.10.016","volume":"20","author":"E Amin","year":"2011","unstructured":"Amin E, Oltean S, Hua J, Gammons M, Hamdollah\u2010Zadeh M, Welsh G, Cheung M\u2010K, Ni L, Kase S, Rennel E et\u00a0al (2011) WT1 mutants reveal SRPK1 to be a downstream angiogenesis target by altering VEGF splicing. 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