{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T04:09:35Z","timestamp":1787630975237,"version":"build-2736575974"},"reference-count":73,"publisher":"American Society for Clinical Investigation","issue":"21","license":[{"start":{"date-parts":[[2023,11,8]],"date-time":"2023-11-08T00:00:00Z","timestamp":1699401600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"crossref","award":["871929"],"award-info":[{"award-number":["871929"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Save Our Sons","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]},{"DOI":"10.13039\/100009358","name":"Duchenne UK","doi-asserted-by":"crossref","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}],"id":[{"id":"10.13039\/100009358","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001784","name":"Victoria University","doi-asserted-by":"crossref","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}],"id":[{"id":"10.13039\/501100001784","id-type":"DOI","asserted-by":"crossref"}]},{"name":"University Children\u2019s Hospital Basel","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,8]]},"abstract":"<jats:p>\n                    New medicines are urgently required to treat the fatal neuromuscular disease Duchenne muscular dystrophy (DMD). Dimethyl fumarate (DMF) is a potent immunomodulatory small molecule nuclear erythroid 2-related factor 2 activator with current clinical utility in the treatment of multiple sclerosis and psoriasis that could be effective for DMD and rapidly translatable. Here, we tested 2 weeks of daily 100 mg\/kg DMF versus 5 mg\/kg standard-care prednisone (PRED) treatment in juvenile\n                    <jats:italic>mdx<\/jats:italic>\n                    mice with early symptomatic DMD. Both drugs modulated seed genes driving the DMD disease program and improved force production in fast-twitch muscle. However, only DMF showed pro-mitochondrial effects, protected contracting muscles from fatigue, improved histopathology, and augmented clinically compatible muscle function tests. DMF may be a more selective modulator of the DMD disease program than PRED, warranting follow-up longitudinal studies to evaluate disease-modifying impact.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.165974","type":"journal-article","created":{"date-parts":[[2023,9,26]],"date-time":"2023-09-26T12:00:38Z","timestamp":1695729638000},"source":"Crossref","is-referenced-by-count":14,"title":["Dimethyl fumarate modulates the dystrophic disease program following short-term treatment"],"prefix":"10.1172","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4567-4319","authenticated-orcid":true,"given":"Cara A.","family":"Timpani","sequence":"first","affiliation":[{"name":"Institute for Health and Sport (IHeS), Victoria University, Melbourne, Victoria, Australia."},{"name":"Australian Institute for Musculoskeletal Science (AIMSS), St Albans, Victoria, Australia."},{"name":"Department of Medicine \u2013 Western Health, Melbourne Medical School, The University of 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