{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T11:15:22Z","timestamp":1788347722747,"version":"build-2803163510"},"reference-count":41,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,10,22]],"date-time":"2021-10-22T00:00:00Z","timestamp":1634860800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009824","name":"FSH Society","doi-asserted-by":"publisher","award":["FSHS\u201022017\u20107"],"award-info":[{"award-number":["FSHS\u201022017\u20107"]}],"id":[{"id":"10.13039\/100009824","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J cachexia sarcopenia muscle"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Facioscapulohumeral dystrophy (FSHD) is caused by mutations leading to the aberrant expression of the DUX4 transcription factor in muscles. DUX4 was proposed to induce cell death, but the involvement of different death pathways is still discussed. A possible pro\u2010apoptotic role of DUX4 was proposed, but as FSHD muscles are characterized by necrosis and inflammatory infiltrates, non\u2010apoptotic pathways may be also involved.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We explored DUX4\u2010mediated cell death by focusing on the role of one regulated necrosis pathway called necroptosis, which is regulated by RIPK3. We investigated the effect of necroptosis on cell death\n                      <jats:italic>in vitro<\/jats:italic>\n                      and\n                      <jats:italic>in vivo<\/jats:italic>\n                      experiments using RIPK3 inhibitors and a RIPK3\u2010deficient transgenic mouse model.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      We showed\n                      <jats:italic>in vitro<\/jats:italic>\n                      that DUX4 expression causes a caspase\u2010independent and RIPK3\u2010mediated cell death in both myoblasts and myotubes.\n                      <jats:italic>In vivo<\/jats:italic>\n                      ,\n                      <jats:italic>RIPK3<\/jats:italic>\n                      \u2010deficient animals present improved body and muscle weights, a reduction of the aberrant activation of the DUX4 network genes, and an improvement of muscle histology.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These results provide evidence for a role of RIPK3 in DUX4\u2010mediated cell death and open new avenues of research.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jcsm.12813","type":"journal-article","created":{"date-parts":[[2021,10,23]],"date-time":"2021-10-23T02:55:24Z","timestamp":1634957724000},"page":"2079-2090","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["RIPK3\u2010mediated cell death is involved in DUX4\u2010mediated toxicity in facioscapulohumeral dystrophy"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3222-9286","authenticated-orcid":false,"given":"Virginie","family":"Mariot","sequence":"first","affiliation":[{"name":"NIHR Biomedical Research Centre University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4902-2035","authenticated-orcid":false,"given":"Romain","family":"Joubert","sequence":"additional","affiliation":[{"name":"NIHR Biomedical Research Centre University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust  London UK"},{"name":"United Kingdom Dementia Research Institute Centre, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience King's College London  London 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 Chamb\u00e9ry France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2239-9094","authenticated-orcid":false,"given":"William","family":"Duddy","sequence":"additional","affiliation":[{"name":"Northern Ireland Center for Stratified\/Personalised Medicine, Biomedical Sciences Research Institute Ulster University  Derry~Londonderry Northern Ireland UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Voit","sequence":"additional","affiliation":[{"name":"NIHR Biomedical Research Centre University College London, Great Ormond Street Institute of Child Health and Great Ormond Street Hospital NHS Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3088-4818","authenticated-orcid":false,"given":"Maximilien","family":"Bencze","sequence":"additional","affiliation":[{"name":"University Paris Est Cr\u00e9teil, INSERM, IMRB  Cr\u00e9teil France"},{"name":"The 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