{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:20:13Z","timestamp":1790832013310,"version":"4.1.0"},"reference-count":134,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2020,7,9]],"date-time":"2020-07-09T00:00:00Z","timestamp":1594252800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Brains to the South","award":["PDR-00351"],"award-info":[{"award-number":["PDR-00351"]}]},{"DOI":"10.13039\/501100007423","name":"Duchenne Parent Project","doi-asserted-by":"publisher","award":["Small starting grant"],"award-info":[{"award-number":["Small starting grant"]}],"id":[{"id":"10.13039\/501100007423","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>The muscle stem cells niche is essential in neuromuscular disorders. Muscle injury and myofiber death are the main triggers of muscle regeneration via satellite cell activation. However, in degenerative diseases such as muscular dystrophy, regeneration still keep elusive. In these pathologies, stem cell loss occurs over time, and missing signals limiting damaged tissue from activating the regenerative process can be envisaged. It is unclear what comes first: the lack of regeneration due to satellite cell defects, their pool exhaustion for degeneration\/regeneration cycles, or the inhibitory mechanisms caused by muscle damage and fibrosis mediators. Herein, Duchenne muscular dystrophy has been taken as a paradigm, as several drugs have been tested at the preclinical and clinical levels, targeting secondary events in the complex pathogenesis derived from lack of dystrophin. We focused on the crucial roles that pro-inflammatory and pro-fibrotic cytokines play in triggering muscle necrosis after damage and stimulating satellite cell activation and self-renewal, along with growth and mechanical factors. These processes contribute to regeneration and niche maintenance. We review the main effects of drugs on regeneration biomarkers to assess whether targeting pathogenic events can help to protect niche homeostasis and enhance regeneration efficiency other than protecting newly formed fibers from further damage.<\/jats:p>","DOI":"10.3390\/cells9071659","type":"journal-article","created":{"date-parts":[[2020,7,10]],"date-time":"2020-07-10T09:45:31Z","timestamp":1594374331000},"page":"1659","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["\u201cThe Social Network\u201d and Muscular Dystrophies: The Lesson Learnt about the Niche Environment as a Target for Therapeutic Strategies"],"prefix":"10.3390","volume":"9","author":[{"given":"Ornella","family":"Cappellari","sequence":"first","affiliation":[{"name":"Section of Pharmacology, Department of Pharmacy-Drug Sciences, University of Bari \u201cAldo Moro\u201d, via Orabona 4\u2014Campus, 70125 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Mantuano","sequence":"additional","affiliation":[{"name":"Section of Pharmacology, Department of Pharmacy-Drug Sciences, University of Bari \u201cAldo Moro\u201d, via Orabona 4\u2014Campus, 70125 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5652-7341","authenticated-orcid":false,"given":"Annamaria","family":"De Luca","sequence":"additional","affiliation":[{"name":"Section of Pharmacology, Department of Pharmacy-Drug Sciences, University of Bari \u201cAldo Moro\u201d, via Orabona 4\u2014Campus, 70125 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,7,9]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1111\/j.1365-2796.2009.02158.x","article-title":"Skeletal muscle stem cells in developmental versus regenerative myogenesis","volume":"266","author":"Tajbakhsh","year":"2009","journal-title":"J. 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