{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T17:36:28Z","timestamp":1787938588495,"version":"build-2784847793"},"reference-count":46,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T00:00:00Z","timestamp":1661299200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"The Association Belge contre les Maladies neuroMusculaires (ABMM)","award":["101034427-2"],"award-info":[{"award-number":["101034427-2"]}]},{"name":"The Association Belge contre les Maladies neuroMusculaires (ABMM)","award":["413501650"],"award-info":[{"award-number":["413501650"]}]},{"name":"European Joint Programme on Rare Diseases (EJP-RD)","award":["101034427-2"],"award-info":[{"award-number":["101034427-2"]}]},{"name":"European Joint Programme on Rare Diseases (EJP-RD)","award":["413501650"],"award-info":[{"award-number":["413501650"]}]},{"name":"Horizon-2020: IMI2-2020-23-05","award":["101034427-2"],"award-info":[{"award-number":["101034427-2"]}]},{"name":"Horizon-2020: IMI2-2020-23-05","award":["413501650"],"award-info":[{"award-number":["413501650"]}]},{"name":"German Academic Exchange Service (DAAD)","award":["101034427-2"],"award-info":[{"award-number":["101034427-2"]}]},{"name":"German Academic Exchange Service (DAAD)","award":["413501650"],"award-info":[{"award-number":["413501650"]}]},{"name":"U4-Society Network OSMYO","award":["101034427-2"],"award-info":[{"award-number":["101034427-2"]}]},{"name":"U4-Society Network OSMYO","award":["413501650"],"award-info":[{"award-number":["413501650"]}]},{"name":"Deutsche Forschungsgemeinschaft (DFG, German Research Foundation)","award":["101034427-2"],"award-info":[{"award-number":["101034427-2"]}]},{"name":"Deutsche Forschungsgemeinschaft (DFG, German Research Foundation)","award":["413501650"],"award-info":[{"award-number":["413501650"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Duchenne Muscular Dystrophy (DMD) is a debilitating muscle disorder that condemns patients to year-long dependency on glucocorticoids. Chronic glucocorticoid use elicits many unfavourable side-effects without offering satisfying clinical improvement, thus, the search for alternative treatments to alleviate muscle inflammation persists. Taurine, an osmolyte with anti-inflammatory effects, mitigated pathological features in the mdx mouse model for DMD but interfered with murine development. In this study, ectoine is evaluated as an alternative for taurine in vitro in CCL-136 cells and in vivo in the mdx mouse. Pre-treating CCL-136 cells with 0.1 mM taurine and 0.1 mM ectoine prior to exposure with 300 U\/mL IFN-\u03b3 and 20 ng\/mL IL-1\u03b2 partially attenuated cell death, whilst 100 mM taurine reduced MHC-I protein levels. In vivo, histopathological features of the tibialis anterior in mdx mice were mitigated by ectoine, but not by taurine. Osmolyte treatment significantly reduced mRNA levels of inflammatory disease biomarkers, respectively, CCL2 and SPP1 in ectoine-treated mdx mice, and CCL2, HSPA1A, TNF-\u03b1 and IL-1\u03b2 in taurine-treated mdx mice. Functional performance was not improved by osmolyte treatment. Furthermore, ectoine-treated mdx mice exhibited reduced body weight. Our results confirmed beneficial effects of taurine in mdx mice and, for the first time, demonstrated similar and differential effects of ectoine.<\/jats:p>","DOI":"10.3390\/ijms23179567","type":"journal-article","created":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T21:03:51Z","timestamp":1661375031000},"page":"9567","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Exploring the Therapeutic Potential of Ectoine in Duchenne Muscular Dystrophy: Comparison with Taurine, a Supplement with Known Beneficial Effects in the mdx Mouse"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9031-2539","authenticated-orcid":false,"given":"Caroline","family":"Merckx","sequence":"first","affiliation":[{"name":"Department of Neurology, Ghent University and Ghent University Hospital, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7062-5319","authenticated-orcid":false,"given":"Jana","family":"Zsch\u00fcntzsch","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie","family":"Meyer","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8939-0169","authenticated-orcid":false,"given":"Robrecht","family":"Raedt","sequence":"additional","affiliation":[{"name":"4BRAIN, Department Head and Skin, Ghent University, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1605-6618","authenticated-orcid":false,"given":"Hanne","family":"Verschuere","sequence":"additional","affiliation":[{"name":"Unit of Molecular Signaling and Cell Death, Center for Inflammation Research, Flemish Institute for Biotechnology, 9052 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Center G\u00f6ttingen, 37075 G\u00f6ttingen, Germany"},{"name":"Department of Neurology and Pain Treatment, Immanuel Klinik R\u00fcdersdorf, University Hospital of the Brandenburg Medical School Theodor Fontane, 15562 R\u00fcdersdorf bei Berlin, Germany"},{"name":"Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, 15562 R\u00fcdersdorf bei Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9403-4401","authenticated-orcid":false,"given":"Boel","family":"De Paepe","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ghent University and Ghent University Hospital, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan L.","family":"De Bleecker","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ghent University and Ghent University Hospital, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3879","DOI":"10.1111\/febs.15466","article-title":"The discovery of dystrophin, the protein product of the Duchenne muscular dystrophy gene","volume":"287","author":"Hoffman","year":"2020","journal-title":"FEBS J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1186\/s13018-022-02996-8","article-title":"Global prevalence of Duchenne and Becker muscular dystrophy: A systematic review and meta-analysis","volume":"17","author":"Salari","year":"2022","journal-title":"J. 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