{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T21:18:43Z","timestamp":1774646323214,"version":"3.50.1"},"reference-count":63,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2022,11,24]],"date-time":"2022-11-24T00:00:00Z","timestamp":1669248000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Associazione Amici Centro Dino Ferrari Fondazione IRCCS C\u00e0 Granda, Ospedale Maggiore Policlinico 5 \u00d7 1000 Research Award"},{"name":"Fondo Europeo di Sviluppo Regionale 2014\u20132020"},{"name":"POR FESR 2014\u20132020, Ricerca Innovazione and Gruppo familiari beta-sarcoglicanopatie"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Muscle wasting is a major pathological feature observed in Duchenne muscular dystrophy (DMD) and is the result of the concerted effects of inflammation, oxidative stress and cell senescence. The inducible form of proteasome, or immunoproteasome (IP), is involved in all the above mentioned processes, regulating antigen presentation, cytokine production and immune cell response. IP inhibition has been previously shown to dampen the altered molecular, histological and functional features of 3-month-old mdx mice, the animal model for DMD. In this study, we described the role of ONX-0914, a selective inhibitor of the PSMB8 subunit of immunoproteasome, in ameliorating the pathological traits that could promote muscle wasting progression in older, 9-month-old mdx mice. ONX-0914 reduces the number of macrophages and effector memory T cells in muscle and spleen, while increasing the number of regulatory T cells. It modulates inflammatory markers both in skeletal and cardiac muscle, possibly counteracting heart remodeling and hypertrophy. Moreover, it buffers oxidative stress by improving mitochondrial efficiency. These changes ultimately lead to a marked decrease of fibrosis and, potentially, to more controlled myofiber degeneration\/regeneration cycles. Therefore, ONX-0914 is a promising molecule that may slow down muscle mass loss, with relatively low side effects, in dystrophic patients with moderate to advanced disease.<\/jats:p>","DOI":"10.3390\/ijms232314657","type":"journal-article","created":{"date-parts":[[2022,11,24]],"date-time":"2022-11-24T03:58:16Z","timestamp":1669262296000},"page":"14657","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Immunoproteasome Inhibition Ameliorates Aged Dystrophic Mouse Muscle Environment"],"prefix":"10.3390","volume":"23","author":[{"given":"Luana","family":"Tripodi","sequence":"first","affiliation":[{"name":"Stem Cell Laboratory, Dino Ferrari Center, Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7536-208X","authenticated-orcid":false,"given":"Davide","family":"Molinaro","sequence":"additional","affiliation":[{"name":"Stem Cell Laboratory, Dino Ferrari Center, Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Fortunato","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology and Transplantation, Universit\u00e0 degli Studi di Milano, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolina","family":"Mella","sequence":"additional","affiliation":[{"name":"Stem Cell Laboratory, Dino Ferrari Center, Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Cassani","sequence":"additional","affiliation":[{"name":"Department of Medical Biotechnologies and Translational Medicine, Universit\u00e0 Degli Studi di Milano, 20089 Milan, Italy"},{"name":"Humanitas Clinical and Research Center IRCCS, Rozzano, 20089 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2705-3984","authenticated-orcid":false,"given":"Yvan","family":"Torrente","sequence":"additional","affiliation":[{"name":"Stem Cell Laboratory, Dino Ferrari Center, Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy"},{"name":"Neurology Unit, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8514-4921","authenticated-orcid":false,"given":"Andrea","family":"Farini","sequence":"additional","affiliation":[{"name":"Neurology Unit, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e1010966","DOI":"10.1080\/21675511.2015.1010966","article-title":"The immune system in Duchenne muscular dystrophy: Friend or foe","volume":"3","author":"Villalta","year":"2015","journal-title":"Rare Dis."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"274","DOI":"10.3389\/fnagi.2014.00274","article-title":"Sarcopenia and sarcopenic obesity in patients with muscular dystrophy","volume":"6","author":"Merlini","year":"2014","journal-title":"Front. 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