{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:03:00Z","timestamp":1791565380483,"version":"4.3.4"},"reference-count":38,"publisher":"The Company of Biologists","issue":"3","license":[{"start":{"date-parts":[[2025,4,2]],"date-time":"2025-04-02T00:00:00Z","timestamp":1743552000000},"content-version":"vor","delay-in-days":32,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100000052","name":"NIH Office of the Director","doi-asserted-by":"crossref","award":["R01HL155907"],"award-info":[{"award-number":["R01HL155907"]}],"id":[{"id":"10.13039\/100000052","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100002108","name":"Friedreich's Ataxia Research Alliance","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100002108","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01HL155907"],"award-info":[{"award-number":["R01HL155907"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R33NS106719"],"award-info":[{"award-number":["R33NS106719"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000009","name":"Foundation for the National Institutes of Health","doi-asserted-by":"crossref","award":["T32 HL086350"],"award-info":[{"award-number":["T32 HL086350"]}],"id":[{"id":"10.13039\/100000009","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100007707","name":"University of California, Davis","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100007707","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Friedreich's ataxia, a recessive disorder caused by a mutation in the frataxin (FXN) gene, has few mouse models that demonstrate a progressive behavioral decline paralleling that of patients. A mouse model of systemic frataxin deficiency, the FXNKD, was recently developed using a doxycycline-inducible method; it is thought to mimic the patient phenotype seen when frataxin levels are decreased, but it has not been determined whether it is reliable for assessment of therapeutics. FXNKD mice underwent testing for 12\u00a0weeks alongside littermates, undergoing tests of motor function, gait and sensation. Additionally, a subset underwent treatment with omaveloxolone or dimethyl fumarate, both NRF2 inducers. We identified multiple techniques that sensitively detect decline in the mice, including open field, gait analysis and Von Frey tests. Furthermore, we developed a novel Salinas\u2013Montgomery ataxia scale, which allows for more comprehensive assessment than a four-part cerebellar ataxia scale. Despite validating multiple sensitive techniques, we did not see any benefits of NRF2-inducing therapies in any tests. This was exacerbated by the discovery of a sexual dimorphism in FXNKD mice, in which males show more significant decline and better responsiveness to NRF2-inducing therapeutics.<\/jats:p>","DOI":"10.1242\/dmm.052128","type":"journal-article","created":{"date-parts":[[2025,2,28]],"date-time":"2025-02-28T09:01:59Z","timestamp":1740733319000},"source":"Crossref","is-referenced-by-count":3,"title":["Robust behavioral assessment of the inducible Friedreich's ataxia mouse does not show improvement with NRF2 induction"],"prefix":"10.1242","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1107-1029","authenticated-orcid":false,"given":"Claire B.","family":"Montgomery","sequence":"first","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-4743-2134","authenticated-orcid":false,"given":"Lili","family":"Salinas","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-9653-8245","authenticated-orcid":false,"given":"Garrett P.","family":"Cox","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-2488-2632","authenticated-orcid":false,"given":"Lauren E.","family":"Adcock","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-7767-5173","authenticated-orcid":false,"given":"Tiffany","family":"Chang","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]},{"name":"Biomedical Research Models Inc. 2 , Richmond, CA 94806 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8089-0657","authenticated-orcid":false,"given":"Francisco","family":"Figueroa","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2563-4133","authenticated-orcid":false,"given":"Gino","family":"Cortopassi","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0601-9816","authenticated-orcid":false,"given":"Elena N.","family":"Dedkova","sequence":"additional","affiliation":[{"name":"University of California 1 Department of Molecular Biosciences , , Davis, Davis, CA 95616 ,","place":["USA"]},{"name":"California Northstate University 3 Department of Basic Sciences , , Elk Grove, CA 95757 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2025,4,2]]},"reference":[{"key":"2025040209321033200_DMM052128C1","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1016\/j.ygeno.2006.06.015","article-title":"GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology","volume":"88","author":"Al-Mahdawi","year":"2006","journal-title":"Genomics"},{"key":"2025040209321033200_DMM052128C2","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1016\/S0887-8994(03)00004-3","article-title":"Friedreich's ataxia","volume":"28","author":"Alper","year":"2003","journal-title":"Pediatr. 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