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Accumulating evidence describes the crucial role of mitochondrial dysfunction and oxidative stress in skeletal muscle of SMA patients. We aimed to investigate the effects of prenatal supplementation with the antioxidant molecule ergothioneine (ERGO) on an SMN\u03947 mouse model of SMA containing a knockout of survival motor neuron protein (SMN1) and two transgenes, one with a single normal copy of human SMN2 and the second with a human SMN2 promoter and a human SMN2 cDNA lacking exon 7. ERGO had a significant positive effect on the survival and locomotor abilities of SMA pups. In isolated diaphragm muscle, ERGO was found to stimulate mitophagy. The results of the current study highlight the need for further research into ERGO as an adjuvant therapy for SMA.<\/jats:p>\n                  <jats:p>\n                    <jats:boxed-text content-type=\"box\" position=\"anchor\">\n                      <jats:caption>\n                        <jats:title>Impact statement<\/jats:title>\n                      <\/jats:caption>\n                      <jats:p>Our finding that ergothioneine supplementation improves survival in a murine model of spinal muscular atrophy may aid research into a novel potential adjuvant to alleviate the symptoms of this serious neuromuscular disease in humans.<\/jats:p>\n                    <\/jats:boxed-text>\n                  <\/jats:p>","DOI":"10.1002\/1873-3468.70136","type":"journal-article","created":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T17:59:26Z","timestamp":1754503166000},"page":"3086-3102","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Ergothioneine supplementation improves pup phenotype and survival in a murine model of spinal muscular atrophy"],"prefix":"10.1002","volume":"599","author":[{"given":"Francesca","family":"Cadile","sequence":"first","affiliation":[{"name":"Department of Molecular Medicine, Via Forlanini 6 University of Pavia  Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Ratto","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnology \u201cL. 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