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This study explores the role of mitochondrial dysfunction as an intramuscular signal for the extracellular matrix (ECM)\u2013based proteolysis and, consequentially, muscle cell dystrophy. We found that inhibition of the mitochondrial electron transport chain causes paralysis as well as muscle structural damage in the nematode\n                    <jats:italic>Caenorhabditis elegans<\/jats:italic>\n                    . This was associated with a significant decline in collagen content. Both paralysis and muscle damage could be rescued with collagen IV overexpression, matrix metalloproteinase (MMP), and Furin inhibitors in Antimycin A\u2013treated animal as well as in the\n                    <jats:italic>C. elegans<\/jats:italic>\n                    Duchenne muscular dystrophy model. Additionally, muscle cytosolic calcium increased in the Antimycin A\u2013treated worms, and its down\u2010regulation rescued the muscle damage, suggesting that calcium overload acts as one of the early triggers and activates Furin and MMPs for collagen degradation. In conclusion, we have established ECM degradation as an important pathway of muscle damage.\u2014Sudevan, S., Takiura, M., Kubota, Y., Higashitani, N., Cooke, M., Ellwood, R. A., Etheridge, T., Szewczyk, N. J., Higashitani, A. Mitochondrial dysfunction causes Ca\n                    <jats:sup>2+<\/jats:sup>\n                    overload and ECM degradation\u2013mediated muscle damage in\n                    <jats:italic>C. elegans<\/jats:italic>\n                    . FASEB J. 33, 9540\u20139550 (2019).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.201802298r","type":"journal-article","created":{"date-parts":[[2019,6,4]],"date-time":"2019-06-04T15:58:50Z","timestamp":1559663930000},"page":"9540-9550","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Mitochondrial dysfunction causes Ca\n                    <sup>2+<\/sup>\n                    overload and ECM degradation\u2013mediated muscle damage in\n                    <i>C. elegans<\/i>"],"prefix":"10.1096","volume":"33","author":[{"given":"Surabhi","family":"Sudevan","sequence":"first","affiliation":[{"name":"Graduate School of Life Sciences Tohoku University  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mai","family":"Takiura","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences Tohoku University  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukihiko","family":"Kubota","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences Tohoku University  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nahoko","family":"Higashitani","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences Tohoku University  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Cooke","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences Tohoku University  Sendai Japan"},{"name":"College of Life and Environmental Science University of Exeter  Exeter United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca A.","family":"Ellwood","sequence":"additional","affiliation":[{"name":"Medical Research Council (MRC) Arthritis Research United Kingdom (ARUK) Centre of Musculoskeletal Ageing Research National Institute for Health Research Nottingham Biomedical Research Centre University of Nottingham  Nottingham United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Etheridge","sequence":"additional","affiliation":[{"name":"College of Life and Environmental Science University of Exeter  Exeter United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathaniel J.","family":"Szewczyk","sequence":"additional","affiliation":[{"name":"Medical Research Council (MRC) Arthritis Research United Kingdom (ARUK) Centre of Musculoskeletal Ageing Research National Institute for Health Research Nottingham Biomedical Research Centre University of Nottingham  Nottingham United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Higashitani","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences Tohoku University  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,6,4]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1515\/hsz-2012-0247"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.2337\/diabetes.51.10.2944"},{"key":"e_1_2_8_4_1","doi-asserted-by":"crossref","first-page":"211","DOI":"10.4236\/ojemd.2014.48020","article-title":"Mitochondrial dysfunction in Duchenne muscular dystrophy","volume":"4","author":"Kelly\u2010Worden M.","year":"2014","journal-title":"Open J. 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