{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T03:57:33Z","timestamp":1789617453164,"version":"build-2803163510"},"reference-count":72,"publisher":"Oxford University Press (OUP)","issue":"15","license":[{"start":{"date-parts":[[2024,5,6]],"date-time":"2024-05-06T00:00:00Z","timestamp":1714953600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Muscular Dystrophy Australia"},{"name":"Murdoch Children\u2019s Research Institute and the Victorian Government\u2019s Operational Infrastructure Support Program"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 AR048179"],"award-info":[{"award-number":["R01 AR048179"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["T32 AR059033"],"award-info":[{"award-number":["T32 AR059033"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["F32 AR069469"],"award-info":[{"award-number":["F32 AR069469"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Muscular Dystrophy Association USA","award":["274143"],"award-info":[{"award-number":["274143"]}]},{"name":"Muscular Dystrophy Association USA","award":["416364"],"award-info":[{"award-number":["416364"]}]},{"name":"National Health and Medical Research Council of Australia","award":["GNT1043837"],"award-info":[{"award-number":["GNT1043837"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,22]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Duchenne Muscular Dystrophy (DMD) is a progressive and fatal neuromuscular disease. Cycles of myofibre degeneration and regeneration are hallmarks of the disease where immune cells infiltrate to repair damaged skeletal muscle. Benfotiamine is a lipid soluble precursor to thiamine, shown clinically to reduce inflammation in diabetic related complications. We assessed whether benfotiamine administration could reduce inflammation related dystrophic pathology. Benfotiamine (10\u00a0mg\/kg\/day) was fed to male mdx mice (n\u2009=\u20097) for 15\u00a0weeks from 4\u00a0weeks of age. Treated mice had an increased growth weight (5\u20137\u00a0weeks) and myofibre size at treatment completion. Markers of dystrophic pathology (area of damaged necrotic tissue, central nuclei) were reduced in benfotiamine mdx quadriceps. Grip strength was increased and improved exercise capacity was found in mdx treated with benfotiamine for 12\u00a0weeks, before being placed into individual cages and allowed access to an exercise wheel for 3\u00a0weeks. Global gene expression profiling (RNAseq) in the gastrocnemius revealed benfotiamine regulated signalling pathways relevant to dystrophic pathology (Inflammatory Response, Myogenesis) and fibrotic gene markers (Col1a1, Col1a2, Col4a5, Col5a2, Col6a2, Col6a2, Col6a3, Lum) towards wildtype levels. In addition, we observed a reduction in gene expression of inflammatory gene markers in the quadriceps (Emr1, Cd163, Cd4, Cd8, Ifng). Overall, these data suggest that benfotiamine reduces dystrophic pathology by acting on inflammatory and fibrotic gene markers and signalling pathways. Given benfotiamine\u2019s excellent safety profile and current clinical use, it could be used in combination with glucocorticoids to treat DMD patients.<\/jats:p>","DOI":"10.1093\/hmg\/ddae066","type":"journal-article","created":{"date-parts":[[2024,5,6]],"date-time":"2024-05-06T23:54:25Z","timestamp":1715039665000},"page":"1339-1355","source":"Crossref","is-referenced-by-count":8,"title":["Benfotiamine improves dystrophic pathology and exercise capacity in <i>mdx<\/i> mice by reducing inflammation and fibrosis"],"prefix":"10.1093","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9774-4089","authenticated-orcid":false,"given":"Chantal A","family":"Coles","sequence":"first","affiliation":[{"name":"Murdoch Childrens Research Institute, The Royal Children\u2019s Hospital , 50 Flemington Road, Parkville, Victoria 3052 , Australia"},{"name":"Faculty of Veterinary and Agricultural Sciences, The University of Melbourne , Flemington Road, Parkville, Victoria 3052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keryn G","family":"Woodman","sequence":"additional","affiliation":[{"name":"Murdoch Childrens Research Institute, The Royal Children\u2019s Hospital , 50 Flemington Road, Parkville, Victoria 3052 , Australia"},{"name":"Faculty of Veterinary and Agricultural Sciences, The University of Melbourne , Flemington Road, Parkville, Victoria 3052 , Australia"},{"name":"Department of Genetics , Yale Medical School, , 333 Cedar Street, New Haven, Connecticut 06520 , USA"},{"name":"Yale University , Yale Medical School, , 333 Cedar Street, New Haven, Connecticut 06520 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth M","family":"Gibbs","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California , 612 Charles E Young Dr S, Los Angeles 90095, California , USA"},{"name":"Center for Duchenne Muscular Dystrophy, University of California , 615 Charles E Young Dr S, Los Angeles 90095, California , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachelle H","family":"Crosbie","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California , 612 Charles E Young Dr S, Los Angeles 90095, California , USA"},{"name":"Center for Duchenne Muscular Dystrophy, University of California , 615 Charles E Young Dr S, Los Angeles 90095, California , USA"},{"name":"Department of Neurology , David Geffen School of Medicine, , 610 Charles E Young Dr S, Los Angeles, California 90095 , USA"},{"name":"University of California , David Geffen School of Medicine, , 610 Charles E Young Dr S, Los Angeles, California 90095 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason D","family":"White","sequence":"additional","affiliation":[{"name":"Murdoch Childrens Research Institute, The Royal Children\u2019s Hospital , 50 Flemington Road, Parkville, Victoria 3052 , Australia"},{"name":"Faculty of Veterinary and Agricultural Sciences, The University of Melbourne , Flemington Road, Parkville, Victoria 3052 , Australia"},{"name":"Charles Sturt University , Office of the Deputy Vice Chancellor Research, Boorooma Street, Wagga Wagga, NSW 2678 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shireen R","family":"Lamand\u00e9","sequence":"additional","affiliation":[{"name":"Murdoch Childrens Research Institute, The Royal Children\u2019s Hospital , 50 Flemington Road, Parkville, Victoria 3052 , Australia"},{"name":"Department of Paediatrics, University of Melbourne , 50 Flemington Road, Parkville, Victoria 3052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,5,6]]},"reference":[{"key":"2024072221204302600_ref1","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1002\/ana.23528","article-title":"Evidence-based path to 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