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Hexanucleotide repeat expansions in the\n                    <jats:italic>C9orf72<\/jats:italic>\n                    gene, the most common genetic cause of ALS (C9\u2010ALS), drive toxicity through different mechanisms. These pathological changes include alterations in stress granules (SGs), ribonucleoprotein complexes formed under stress conditions. Here, we show that G3BP1, a core component of SGs, exhibits enhanced interaction with the nucleoporin Nup107 in motor neurons derived from patient iPSCs carrying\n                    <jats:italic>C9orf72<\/jats:italic>\n                    mutations. Moreover, Nup107 colocalizes with SGs and aggregates in C9\u2010ALS motor neurons. Notably, knockdown of\n                    <jats:italic>npp\u20105<\/jats:italic>\n                    , the\n                    <jats:italic>Caenorhabditis elegans<\/jats:italic>\n                    ortholog of\n                    <jats:italic>Nup107,<\/jats:italic>\n                    alleviates ALS\u2010associated phenotypes in worm models, including reduced lifespan and impaired motility. Together, our findings provide insights into disease\u2010related changes in C9\u2010ALS pathogenesis.\n                  <\/jats:p>","DOI":"10.1002\/1873-3468.70156","type":"journal-article","created":{"date-parts":[[2025,9,2]],"date-time":"2025-09-02T06:00:09Z","timestamp":1756792809000},"page":"3047-3065","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["<i>C9orf72<\/i>\n                    <scp>ALS<\/scp>\n                    \u2010causing mutations lead to mislocalization and aggregation of nucleoporin Nup107 into stress granules"],"prefix":"10.1002","volume":"599","author":[{"given":"Sayg\u0131n","family":"Bilican","sequence":"first","affiliation":[{"name":"Institute for Integrated Stress Response Signaling, Faculty of Medicine University Hospital Cologne  Germany"},{"name":"Cologne Excellence Cluster for Cellular Stress Responses in Aging\u2010Associated Diseases (CECAD) University of Cologne  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