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Mouse models based on TDP-43 are thus\u00a0essential for studying ALS and developing therapeutic approaches. The TDP-43 rNLS8 mouse model expresses a human TDP-43 transgene with a mutated nuclear localization sequence (hTDP-43 \u0394NLS), but this\u00a0is normally suppressed by the presence of doxycycline (Dox). Disease is initiated by removal of Dox,\u00a0which\u00a0replicates key ALS features, including TDP-43 pathology, neuromuscular junction denervation, motor neuron loss, and reduced survival. However, this model\u00a0has a\u00a0rapid disease progression which\u00a0limits its use for extended preclinical studies\u00a0and investigation\u00a0of\u00a0early disease mechanisms. To overcome these limitations, we explored whether maintaining low Dox concentrations in the diet (10\u201320\u00a0mg\/kg) could slow disease progression. Our findings demonstrate that this approach significantly\u00a0reduced hTDP-43 \u0394NLS expression (up to 4.8-fold), which\u00a0delayed disease onset by four weeks. Disease progression, assessed by rotarod performance, grip strength, and neurological scores, was\u00a0extended from six to 15\u00a0weeks, with a threefold increase in survival. Despite slower progression, at the end stage, mice displayed similar levels of\u00a0neuroinflammation, motor neuron loss, as Dox off mice. These findings highlight slower-progressing TDP-43 rNLS8 mice as a robust model for preclinical and early disease mechanism studies.<\/jats:p>","DOI":"10.1007\/s12017-025-08871-z","type":"journal-article","created":{"date-parts":[[2025,8,18]],"date-time":"2025-08-18T09:23:48Z","timestamp":1755509028000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["A Slower-Progressing TDP-43 rNLS8 Mouse Model for ALS: Implications for Preclinical and Mechanistic Studies"],"prefix":"10.1007","volume":"27","author":[{"given":"Cyril Jones","family":"Jagaraj","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prachi","family":"Mehta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Hunter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie D.","family":"Atkin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,18]]},"reference":[{"issue":"3","key":"8871_CR1","doi-asserted-by":"publisher","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","volume":"351","author":"T Arai","year":"2006","unstructured":"Arai, T., Hasegawa, M., Akiyama, H., Ikeda, K., Nonaka, T., Mori, H., & Oda, T. 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