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The deregulated molecular network causing FSHD muscle dysfunction and pathology is not well understood. It has been shown that the hypoxia response factor HIF1\u03b1 is critically disturbed in FSHD and has a major role in DUX4-induced cell death. In this study, we further explored the relationship between DUX4 and HIF1\u03b1. We found that the DUX4 and HIF1\u03b1 link differed according to the stage of myogenic differentiation and was conserved between human and mouse muscle. Furthermore, we found that HIF1\u03b1 knockdown in a mouse model of DUX4 local expression exacerbated DUX4-mediated muscle fibrosis. Our data indicate that the suggested role of HIF1\u03b1 in DUX4 toxicity is complex and that targeting HIF1\u03b1 might be challenging in the context of FSHD therapeutic approaches.<\/jats:p>","DOI":"10.3390\/ijms25063327","type":"journal-article","created":{"date-parts":[[2024,3,15]],"date-time":"2024-03-15T09:32:30Z","timestamp":1710495150000},"page":"3327","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["The DUX4\u2013HIF1\u03b1 Axis in Murine and Human Muscle Cells: A Link More Complex Than Expected"],"prefix":"10.3390","volume":"25","author":[{"given":"Thuy-Hang","family":"Nguyen","sequence":"first","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maelle","family":"Limpens","sequence":"additional","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sihame","family":"Bouhmidi","sequence":"additional","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lise","family":"Paprzycki","sequence":"additional","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Legrand","sequence":"additional","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0838-2432","authenticated-orcid":false,"given":"Anne-Emilie","family":"Decl\u00e8ves","sequence":"additional","affiliation":[{"name":"Department of Metabolic and Molecular Biochemistry, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp","family":"Heher","sequence":"additional","affiliation":[{"name":"Randall Centre for Cell and Molecular Biophysics, King\u2019s College London, Guy\u2019s Campus, London SE1 1UL, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5115-2424","authenticated-orcid":false,"given":"Alexandra","family":"Belayew","sequence":"additional","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher R. S.","family":"Banerji","sequence":"additional","affiliation":[{"name":"Randall Centre for Cell and Molecular Biophysics, King\u2019s College London, Guy\u2019s Campus, London SE1 1UL, UK"},{"name":"The Alan Turing Institute, The British Library, London NW1 2DB, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9562-3072","authenticated-orcid":false,"given":"Peter S.","family":"Zammit","sequence":"additional","affiliation":[{"name":"Randall Centre for Cell and Molecular Biophysics, King\u2019s College London, Guy\u2019s Campus, London SE1 1UL, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2154-7529","authenticated-orcid":false,"given":"Alexandra","family":"Tassin","sequence":"additional","affiliation":[{"name":"Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,3,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e13695","DOI":"10.15252\/emmm.202013695","article-title":"Pathomechanisms and Biomarkers in Facioscapulohumeral Muscular Dystrophy: Roles of DUX4 and PAX7","volume":"13","author":"Banerji","year":"2021","journal-title":"EMBO Mol. 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