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As RNA editing alters the sequence of the RNA, it can affect splicing, stability, miRNA binding and may also lead to recoding of the translated protein. Recently, we analysed recoding A-to-I RNA editing in chronic lymphocytic leukaemia (CLL) and could define prognostically relevant editing patterns. However, disease relevant C-to-U RNA editing in CLL remained unexplored. In this study, we examined C-to-U RNA editing in CLL and discovered a recoding RNA editing site within the <jats:italic>MFN1<\/jats:italic> gene (hg38; chr3:179,375,230), which has recently been described as RNA editing site in brain samples. We found that <jats:italic>MFN1<\/jats:italic> editing was not only present in CLL samples but also in naive B cell subsets, primarily occurred at unspliced RNA and correlated with intron retention. We further identified catalytically active ADARB1 as an essential regulator for <jats:italic>MFN1<\/jats:italic> editing. Finally, <jats:italic>MFN1<\/jats:italic> editing correlated with prolonged time to treatment and overall survival in CLL patients. Summarizing, we identified a novel ADARB1 function as C to U editing regulator, which regulates <jats:italic>MFN1<\/jats:italic> splicing and MFN1 S329L recoding with pathogenic relevance in CLL.<\/jats:p>","DOI":"10.1038\/s41598-025-15666-6","type":"journal-article","created":{"date-parts":[[2025,8,14]],"date-time":"2025-08-14T14:56:04Z","timestamp":1755183364000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["C to U RNA editing of MFN1 is regulated by ADARB1 and associates with favourable prognosis in chronic lymphocytic leukemia"],"prefix":"10.1038","volume":"15","author":[{"given":"Alejandra","family":"Gonzalez Martinez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franz Josef","family":"Gassner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dominik","family":"Baumgartner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philine","family":"Hoven","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ekaterina","family":"Akimova","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markus","family":"Steiner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephanie","family":"Schlichtner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefanie","family":"Rauscher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ferran","family":"Nadeu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elias","family":"Campo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard","family":"Greil","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander","family":"Egle","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa","family":"Pleyer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nadja","family":"Zaborsky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roland","family":"Geisberger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,8,14]]},"reference":[{"key":"15666_CR1","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1038\/s41576-018-0006-1","volume":"19","author":"E Eisenberg","year":"2018","unstructured":"Eisenberg, E. & Levanon, E. 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