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There is general consensus that recognition of extrahelical extrusions or hairpin-loop structures (formed by strand slippage) by the DNA mismatch repair protein MutS\u03b2 leads to repeat expansion by a mutagenic process. By contrast, the FAN1 nuclease attenuates triplet repeat expansion, the molecular basis of which was explained by our recent finding that FAN1 nuclease cleaves and initiates removal of extrahelical extrusions. Here we show that extrusions containing two or more triplet repeats are subject to recognition and processing by either FAN1 or MutS\u03b2. However, extrusions containing a single triplet escape FAN1 cleavage and are preferentially processed by a MutS\u03b2-dependent process, leading to repeat expansion. Thus, extrahelical extrusion size determines the ultimate fate of the repeat element, the protective role of FAN1 being limited to removal of extrusions containing two or more triplets. Therefore, repeat expansion is a net consequence of MutS\u03b2-dependent processing of single triplet extrusions and competition between MutS\u03b2 and FAN1 for extrusions containing two or more triplets. These findings provide new insights into the role of DNA structural dynamics in determining pathway choice in DNArepair.<\/jats:p>","DOI":"10.1093\/nar\/gkaf1393","type":"journal-article","created":{"date-parts":[[2025,11,25]],"date-time":"2025-11-25T12:57:29Z","timestamp":1764075449000},"source":"Crossref","is-referenced-by-count":4,"title":["DNA extrusion size determines pathway choice during CAG repeat expansion"],"prefix":"10.1093","volume":"53","author":[{"given":"Mayuri","family":"Bhatia","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Sidney Kimmel Medical College, Thomas Jefferson University , Philadelphia, PA 19107 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashutosh S","family":"Phadte","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Sidney Kimmel Medical College, Thomas Jefferson University , Philadelphia, PA 19107 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