{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T17:46:48Z","timestamp":1775065608692,"version":"3.50.1"},"reference-count":29,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2025,5,26]],"date-time":"2025-05-26T00:00:00Z","timestamp":1748217600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001602","name":"Science Foundation Ireland","doi-asserted-by":"publisher","award":["SFI 19\/FFP\/6628 INSPIRE DMD"],"award-info":[{"award-number":["SFI 19\/FFP\/6628 INSPIRE DMD"]}],"id":[{"id":"10.13039\/501100001602","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["physoc.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Physiology"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Duchenne muscular dystrophy (DMD) is characterized by respiratory muscle injury and weakness, ultimately leading to respiratory failure. Impaired respiratory muscle performance, fibrosis and inflammation in early disease are evident in the dystrophin\u2010deficient\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse model of DMD. Prednisone or similar treatment is the current standard of care for DMD and exerts its benefits via an anti\u2010inflammatory action, but chronic treatment is associated with side\u2010effects. A recent study demonstrated improved function in\n                    <jats:italic>mdx<\/jats:italic>\n                    limb muscle with weekly glucocorticoid treatment compared with daily treatment. Herein, we investigated the effect of weekly \u03b1\u2010methylprednisolone (PRED) treatment alone and the effect\u00a0of PRED in combination with daily intake of the antioxidant\n                    <jats:italic>N<\/jats:italic>\n                    \u2010acetyl cysteine, NAC (PREDNAC) on respiratory performance. One\u2010month\u2010old male\n                    <jats:italic>mdx<\/jats:italic>\n                    \u00a0mice received PRED (0.8\u00a0mg\/kg methylprednisolone\n                    <jats:sc>i.p<\/jats:sc>\n                    . weekly) or PREDNAC (0.8\u00a0mg\/kg methylprednisolone\n                    <jats:sc>i.p<\/jats:sc>\n                    . weekly and 1% NAC in drinking water daily) for 3 months. At 4 months of age, conscious breathing was measured in vivo by whole\u2010body plethysmography. Under urethane general anaesthesia, respiratory EMG and inspiratory pressure were measured at baseline and during maximal activity. The intrinsic force\u2010generating capacity of the diaphragm was determined\n                    <jats:italic>ex vivo<\/jats:italic>\n                    . Neither PRED nor PREDNAC influenced breathing or diaphragm force\u2010generating capacity in\n                    <jats:italic>mdx<\/jats:italic>\n                    mice. There was a significant increase in diaphragm and parasternal EMG activity, but inspiratory pressure was unchanged with treatment. We conclude that neither PRED nor PREDNAC has a major beneficial effect on respiratory system performance in the\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse model of DMD. Weekly administration of glucocorticoids is inadequate to protect respiratory performance in\n                    <jats:italic>mdx<\/jats:italic>\n                    mice, which might reflect the higher duty cycle of respiratory muscles compared with limb muscles.\n                  <\/jats:p>","DOI":"10.1113\/ep092491","type":"journal-article","created":{"date-parts":[[2025,5,26]],"date-time":"2025-05-26T12:40:33Z","timestamp":1748263233000},"page":"2019-2035","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Exploring the respiratory efficacy of combined chronic glucocorticoid and antioxidant interventions in the\n                    <i>mdx<\/i>\n                    mouse: The PREDNAC trial"],"prefix":"10.1113","volume":"110","author":[{"given":"Michael N.","family":"Maxwell","sequence":"first","affiliation":[{"name":"Department of Physiology University College Cork  Cork Ireland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ben T.","family":"Murphy","sequence":"additional","affiliation":[{"name":"Department of Physiology University College Cork  Cork Ireland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fiona B.","family":"McDonald","sequence":"additional","affiliation":[{"name":"Department of Physiology University College Cork  Cork Ireland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken D.","family":"O'Halloran","sequence":"additional","affiliation":[{"name":"Department of Physiology University College Cork  Cork 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of survival in Duchenne Muscular Dystrophy: Retrospective analysis of 835 patients","volume":"31","author":"Passamano L.","year":"2012","journal-title":"Acta Myologica"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2006.01.002"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1113\/JP274229"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI91445"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2023.1109587"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1113\/expphysiol.2013.076828"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.96.6.1346"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.3390\/antiox10060967"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2011.11.007"}],"container-title":["Experimental 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