{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T21:05:28Z","timestamp":1792789528725,"version":"4.5.0"},"reference-count":80,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T00:00:00Z","timestamp":1753920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001459","name":"Government of Singapore Ministry of Education","doi-asserted-by":"publisher","award":["T2EP30122-0015"],"award-info":[{"award-number":["T2EP30122-0015"]}],"id":[{"id":"10.13039\/501100001459","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Current Opinion in Cell Biology"],"published-print":{"date-parts":[[2025,10]]},"DOI":"10.1016\/j.ceb.2025.102579","type":"journal-article","created":{"date-parts":[[2025,8,19]],"date-time":"2025-08-19T16:07:20Z","timestamp":1755619640000},"page":"102579","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"special_numbering":"C","title":["Epigenetic regulation of DUX4: From embryogenesis to muscular degeneration"],"prefix":"10.1016","volume":"96","author":[{"given":"Leyi","family":"Li","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnab","family":"Ray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4668-5952","authenticated-orcid":false,"given":"Shifeng","family":"Xue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ceb.2025.102579_bib1","doi-asserted-by":"crossref","DOI":"10.1387\/ijdb.230247sn","article-title":"DUX4, the rockstar of embryonic genome activation?","author":"Nyk\u00e4nen","year":"2024","journal-title":"Int J Dev Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib2","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1038\/ng.3858","article-title":"DUX-family transcription factors regulate zygotic genome activation in placental mammals","volume":"49","author":"De Iaco","year":"2017","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib3","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1038\/ng.3844","article-title":"Conserved roles of mouse DUX and human DUX4 in activating cleavage-stage genes and MERVL\/HERVL retrotransposons","volume":"49","author":"Hendrickson","year":"2017","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib4","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1038\/ng.3846","article-title":"Conservation and innovation in the DUX4-family gene network","volume":"49","author":"Whiddon","year":"2017","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib5","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0160022","article-title":"Influence of repressive histone and DNA methylation upon D4Z4 transcription in non-myogenic cells","volume":"11","author":"Das","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.ceb.2025.102579_bib6","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1016\/j.devcel.2019.06.011","article-title":"DUX4 suppresses MHC class I to promote cancer immune evasion and resistance to checkpoint blockade","volume":"50","author":"Chew","year":"2019","journal-title":"Dev Cell"},{"key":"10.1016\/j.ceb.2025.102579_bib7","doi-asserted-by":"crossref","first-page":"1650","DOI":"10.1126\/science.1189044","article-title":"A unifying genetic model for facioscapulohumeral muscular dystrophy","volume":"329","author":"Lemmers","year":"2010","journal-title":"Science"},{"key":"10.1016\/j.ceb.2025.102579_bib8","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1016\/j.nmd.2007.04.002","article-title":"The DUX4 gene at the FSHD1A locus encodes a pro-apoptotic protein","volume":"17","author":"Kowaljow","year":"2007","journal-title":"Neuromuscul Disord"},{"key":"10.1016\/j.ceb.2025.102579_bib9","doi-asserted-by":"crossref","first-page":"5901","DOI":"10.1093\/hmg\/ddv315","article-title":"Endogenous DUX4 expression in FSHD myotubes is sufficient to cause cell death and disrupts RNA splicing and cell migration pathways","volume":"24","author":"Rickard","year":"2015","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib10","doi-asserted-by":"crossref","first-page":"2895","DOI":"10.1093\/hmg\/ddg327","article-title":"Expression profiling of FSHD muscle supports a defect in specific stages of myogenic differentiation","volume":"12","author":"Winokur","year":"2003","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib11","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1038\/ng1262","article-title":"Hypomethylation of D4Z4 in 4q-linked and non-4q-linked facioscapulohumeral muscular dystrophy","volume":"35","author":"van Overveld","year":"2003","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib12","doi-asserted-by":"crossref","first-page":"1020","DOI":"10.1016\/j.ajhg.2016.03.013","article-title":"Mutations in DNMT3B modify epigenetic repression of the D4Z4 repeat and the penetrance of facioscapulohumeral dystrophy","volume":"98","author":"van den Boogaard","year":"2016","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib13","doi-asserted-by":"crossref","first-page":"1370","DOI":"10.1038\/ng.2454","article-title":"Digenic inheritance of an SMCHD1 mutation and an FSHD-permissive D4Z4 allele causes facioscapulohumeral muscular dystrophy type 2","volume":"44","author":"Lemmers","year":"2012","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib14","doi-asserted-by":"crossref","first-page":"e2441","DOI":"10.1212\/WNL.0000000000009617","article-title":"Homozygous nonsense variant in LRIF1 associated with facioscapulohumeral muscular dystrophy","volume":"94","author":"Hamanaka","year":"2020","journal-title":"Neurology"},{"key":"10.1016\/j.ceb.2025.102579_bib15","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1002\/humu.21091","article-title":"Common epigenetic changes of D4Z4 in contraction-dependent and contraction-independent FSHD","volume":"30","author":"de Greef","year":"2009","journal-title":"Hum Mutat"},{"key":"10.1016\/j.ceb.2025.102579_bib16","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1000559","article-title":"Specific loss of histone H3 lysine 9 trimethylation and HP1gamma\/cohesin binding at D4Z4 repeats is associated with facioscapulohumeral dystrophy (FSHD)","volume":"5","author":"Zeng","year":"2009","journal-title":"PLoS Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib17","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/j.tig.2017.01.007","article-title":"The epigenetic regulator SMCHD1 in development and disease","volume":"33","author":"Jansz","year":"2017","journal-title":"Trends Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib18","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1093\/hmg\/ddu486","article-title":"Inter-individual differences in CpG methylation at D4Z4 correlate with clinical variability in FSHD1 and FSHD2","volume":"24","author":"Lemmers","year":"2015","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib19","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.abb9149","article-title":"The chromosomal protein SMCHD1 regulates DNA methylation and the 2c-like state of embryonic stem cells by antagonizing TET proteins","volume":"7","author":"Huang","year":"2021","journal-title":"Sci Adv"},{"key":"10.1016\/j.ceb.2025.102579_sref20","doi-asserted-by":"crossref","DOI":"10.1038\/s42003-023-05053-0","article-title":"SMCHD1 and LRIF1 converge at the FSHD-associated D4Z4 repeat and LRIF1 promoter yet display different modes of action","volume":"6","author":"\u0160ikrov\u00e1","year":"2023","journal-title":"Commun Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib21","doi-asserted-by":"crossref","first-page":"2822","DOI":"10.1093\/nar\/gkz005","article-title":"SMCHD1 is involved in de novo methylation of the DUX4-encoding D4Z4 macrosatellite","volume":"47","author":"Dion","year":"2019","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.ceb.2025.102579_bib22","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.cell.2018.05.007","article-title":"SMCHD1 merges chromosome compartments and assists formation of super-structures on the inactive X","volume":"174","author":"Wang","year":"2018","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2025.102579_bib23","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1038\/s41594-018-0111-z","article-title":"Smchd1 regulates long-range chromatin interactions on the inactive X chromosome and at Hox clusters","volume":"25","author":"Jansz","year":"2018","journal-title":"Nat Struct Mol Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib24","article-title":"SMCHD1 compacts DNA directly in an ATP-regulated manner","author":"Ng","year":"2025","journal-title":"bioRxiv"},{"key":"10.1016\/j.ceb.2025.102579_sref25","doi-asserted-by":"crossref","DOI":"10.1016\/j.isci.2024.109357","article-title":"Engineered FSHD mutations results in D4Z4 heterochromatin disruption and feedforward DUX4 network activation","volume":"27","author":"Kong","year":"2024","journal-title":"iScience"},{"key":"10.1016\/j.ceb.2025.102579_bib26","doi-asserted-by":"crossref","first-page":"4053","DOI":"10.1128\/MCB.00432-15","article-title":"Independent mechanisms target SMCHD1 to trimethylated histone H3 lysine 9-modified chromatin and the inactive X chromosome","volume":"35","author":"Brideau","year":"2015","journal-title":"Mol Cell Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib27","doi-asserted-by":"crossref","first-page":"9450","DOI":"10.1093\/nar\/gkae600","article-title":"SMCHD1 activates the expression of genes required for the expansion of human myoblasts","volume":"52","author":"Wong","year":"2024","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.ceb.2025.102579_bib28","doi-asserted-by":"crossref","first-page":"3583","DOI":"10.1038\/s41467-022-31185-8","article-title":"HOX epimutations driven by maternal SMCHD1\/LRIF1 haploinsufficiency trigger homeotic transformations in genetically wildtype offspring","volume":"13","author":"Xue","year":"2022","journal-title":"Nat Commun"},{"key":"10.1016\/j.ceb.2025.102579_bib29","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.adn7732","article-title":"DNMT3B splicing dysregulation mediated by SMCHD1 loss contributes to DUX4 overexpression and FSHD pathogenesis","volume":"10","author":"Engal","year":"2024","journal-title":"Sci Adv"},{"key":"10.1016\/j.ceb.2025.102579_bib30","first-page":"753","article-title":"Polycomb repressive complex 1 provides a molecular explanation for repeat copy number dependency in FSHD muscular dystrophy","volume":"26","author":"Casa","year":"2017","journal-title":"Human Mol Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib31","doi-asserted-by":"crossref","DOI":"10.1186\/s13072-018-0215-z","article-title":"Sporadic DUX4 expression in FSHD myocytes is associated with incomplete repression by the PRC2 complex and gain of H3K9 acetylation on the contracted D4Z4 allele","volume":"11","author":"Haynes","year":"2018","journal-title":"Epigenetics Chromatin"},{"key":"10.1016\/j.ceb.2025.102579_bib32","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/j.cell.2012.03.035","article-title":"A long ncRNA links copy number variation to a polycomb\/trithorax epigenetic switch in FSHD muscular dystrophy","volume":"149","author":"Cabianca","year":"2012","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2025.102579_sref33","doi-asserted-by":"crossref","first-page":"5144","DOI":"10.1093\/nar\/gkad230","article-title":"WDR5 is required for DUX4 expression and its pathological effects in FSHD muscular dystrophy","volume":"51","author":"Mocciaro","year":"2023","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.ceb.2025.102579_bib34","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1080\/15592294.2015.1113798","article-title":"Increased DUX4 expression during muscle differentiation correlates with decreased SMCHD1 protein levels at D4Z4","volume":"10","author":"Balog","year":"2015","journal-title":"Epigenetics"},{"key":"10.1016\/j.ceb.2025.102579_bib35","doi-asserted-by":"crossref","DOI":"10.1186\/s13395-017-0134-x","article-title":"BET bromodomain inhibitors and agonists of the beta-2 adrenergic receptor identified in screens for compounds that inhibit DUX4 expression in FSHD muscle cells","volume":"7","author":"Campbell","year":"2017","journal-title":"Skeletal Muscle"},{"key":"10.1016\/j.ceb.2025.102579_bib36","doi-asserted-by":"crossref","first-page":"11837","DOI":"10.1074\/jbc.RA118.002633","article-title":"Protein kinase A activation inhibits DUX4 gene expression in myotubes from patients with facioscapulohumeral muscular dystrophy","volume":"293","author":"Cruz","year":"2018","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ceb.2025.102579_bib37","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1124\/jpet.119.259663","article-title":"Clinically advanced p38 inhibitors suppress DUX4 expression in cellular and animal models of facioscapulohumeral muscular dystrophy","volume":"370","author":"Oliva","year":"2019","journal-title":"J Pharmacol Exp Therapeut"},{"key":"10.1016\/j.ceb.2025.102579_bib38","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1038\/ng1378","article-title":"p38 pathway targets SWI-SNF chromatin-remodeling complex to muscle-specific loci","volume":"36","author":"Simone","year":"2004","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib39","doi-asserted-by":"crossref","DOI":"10.1016\/j.nmd.2025.105422","article-title":"The recent clinical trial of losmapimod for the treatment of facioscapulohumeral muscular dystrophy","volume":"52","author":"Bloch","year":"2025","journal-title":"Neuromuscul Disord"},{"key":"10.1016\/j.ceb.2025.102579_bib40","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-024-77911-8","article-title":"Temporal variation in p38-mediated regulation of DUX4 in facioscapulohumeral muscular dystrophy","volume":"14","author":"Vangipurapu","year":"2024","journal-title":"Sci Rep"},{"key":"10.1016\/j.ceb.2025.102579_bib41","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1016\/j.ymthe.2018.04.019","article-title":"Identification of epigenetic regulators of DUX4-fl for targeted therapy of facioscapulohumeral muscular dystrophy","volume":"26","author":"Himeda","year":"2018","journal-title":"Mol Ther"},{"key":"10.1016\/j.ceb.2025.102579_bib42","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1038\/s41392-023-01647-6","article-title":"Bromodomain and extraterminal (BET) proteins: biological functions, diseases, and targeted therapy","volume":"8","author":"Wang","year":"2023","journal-title":"Signal Transduct Targeted Ther"},{"key":"10.1016\/j.ceb.2025.102579_bib43","doi-asserted-by":"crossref","first-page":"2683","DOI":"10.3390\/biomedicines11102683","article-title":"Apabetalone, a clinical-stage, selective BET inhibitor, opposes DUX4 target gene expression in primary human FSHD muscle cells","volume":"11","author":"Sarsons","year":"2023","journal-title":"Biomedicines"},{"key":"10.1016\/j.ceb.2025.102579_bib44","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/j.semcdb.2020.03.003","article-title":"Myogenesis control by SIX transcriptional complexes","volume":"104","author":"Maire","year":"2020","journal-title":"Semin Cell Dev Biol"},{"key":"10.1016\/j.ceb.2025.102579_sref45","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1186\/s13395-024-00361-3","article-title":"SIX transcription factors are necessary for the activation of DUX4 expression in facioscapulohumeral muscular dystrophy","volume":"14","author":"Fox","year":"2024","journal-title":"Skeletal Muscle"},{"key":"10.1016\/j.ceb.2025.102579_bib46","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/S1534-5807(01)00011-9","article-title":"Eyes absent mediates cross-talk between retinal determination genes and the receptor tyrosine kinase signaling pathway","volume":"1","author":"Hsiao","year":"2001","journal-title":"Dev Cell"},{"key":"10.1016\/j.ceb.2025.102579_bib47","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms11790","article-title":"Identification of ETV6-RUNX1-like and DUX4-rearranged subtypes in paediatric B-cell precursor acute lymphoblastic leukaemia","volume":"7","author":"Lilljebj\u00f6rn","year":"2016","journal-title":"Nat Commun"},{"key":"10.1016\/j.ceb.2025.102579_bib48","doi-asserted-by":"crossref","first-page":"2125","DOI":"10.1093\/hmg\/ddl136","article-title":"Fusion between CIC and DUX4 up-regulates PEA3 family genes in Ewing-like sarcomas with t(4;19)(q35;q13) translocation","volume":"15","author":"Kawamura-Saito","year":"2006","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ceb.2025.102579_sref49","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2023.113114","article-title":"DUX4 expression in cancer induces a metastable early embryonic totipotent program","volume":"42","author":"Smith","year":"2023","journal-title":"Cell Rep"},{"key":"10.1016\/j.ceb.2025.102579_bib50","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.89017","article-title":"DUX4 is a common driver of immune evasion and immunotherapy failure in metastatic cancers","volume":"12","author":"Pineda","year":"2024","journal-title":"eLife"},{"key":"10.1016\/j.ceb.2025.102579_bib51","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1097\/PAS.0000000000000846","article-title":"Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases","volume":"41","author":"Antonescu","year":"2017","journal-title":"Am J Surg Pathol"},{"key":"10.1016\/j.ceb.2025.102579_bib52","doi-asserted-by":"crossref","first-page":"1469","DOI":"10.1016\/j.celrep.2019.09.087","article-title":"NFE2L3 controls colon cancer cell growth through regulation of DUX4, a CDK1 inhibitor","volume":"29","author":"Bury","year":"2019","journal-title":"Cell Rep"},{"key":"10.1016\/j.ceb.2025.102579_bib53","doi-asserted-by":"crossref","DOI":"10.1172\/JCI141908","article-title":"ETV4 and ETV5 drive synovial sarcoma through cell cycle and DUX4 embryonic pathway control","volume":"131","author":"DeSalvo","year":"2021","journal-title":"J Clin Investig"},{"key":"10.1016\/j.ceb.2025.102579_bib54","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.31023","article-title":"NuRD and CAF-1-mediated silencing of the D4Z4 array is modulated by DUX4-induced MBD3L proteins","volume":"7","author":"Campbell","year":"2018","journal-title":"eLife"},{"key":"10.1016\/j.ceb.2025.102579_bib55","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1038\/s41564-018-0285-5","article-title":"Centrosomal protein TRIM43 restricts herpesvirus infection by regulating nuclear lamina integrity","volume":"4","author":"Full","year":"2019","journal-title":"Nat Microbiol"},{"key":"10.1016\/j.ceb.2025.102579_sref56","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1038\/s41467-025-55928-5","article-title":"Herpesviruses mimic zygotic genome activation to promote viral replication","volume":"16","author":"Neugebauer","year":"2025","journal-title":"Nat Commun"},{"key":"10.1016\/j.ceb.2025.102579_bib57","article-title":"DUX4-stimulated genes define the antiviral response to herpesviruses in human trophoblasts","author":"Hatterschide","year":"2025","journal-title":"bioRxiv"},{"key":"10.1016\/j.ceb.2025.102579_sref58","article-title":"Telomeres cooperate in zygotic genome activation by affecting DUX4\/Dux transcription","volume":"26","author":"Zhang","year":"2023","journal-title":"iScience"},{"key":"10.1016\/j.ceb.2025.102579_bib59","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1038\/nsmb.2571","article-title":"Telomere position effect regulates DUX4 in human facioscapulohumeral muscular dystrophy","volume":"20","author":"Stadler","year":"2013","journal-title":"Nat Struct Mol Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib60","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1038\/s41588-021-00893-0","article-title":"p53 convergently activates Dux\/DUX4 in embryonic stem cells and in facioscapulohumeral muscular dystrophy cell models","volume":"53","author":"Grow","year":"2021","journal-title":"Nat Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib61","doi-asserted-by":"crossref","DOI":"10.1016\/j.bbadis.2022.166367","article-title":"The evolution of DUX4 gene regulation and its implication for facioscapulohumeral muscular dystrophy","volume":"1868","author":"Jagannathan","year":"2022","journal-title":"Biochim Biophys Acta Mol Basis Dis"},{"key":"10.1016\/j.ceb.2025.102579_bib62","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1086\/519311","article-title":"Evolutionary conservation of a coding function for D4Z4, the tandem DNA repeat mutated in facioscapulohumeral muscular dystrophy","volume":"81","author":"Clapp","year":"2007","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib63","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2421594122","article-title":"Dux cluster duplication ensures full activation of totipotent genes","volume":"122","author":"Lin","year":"2025","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.ceb.2025.102579_bib64","first-page":"4577","article-title":"Mouse Dux is myotoxic and shares partial functional homology with its human paralog DUX4","volume":"25","author":"Eidahl","year":"2016","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ceb.2025.102579_bib65","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1038\/nature11244","article-title":"Embryonic stem cell potency fluctuates with endogenous retrovirus activity","volume":"487","author":"Macfarlan","year":"2012","journal-title":"Nature"},{"key":"10.1016\/j.ceb.2025.102579_bib66","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pbio.3000324","article-title":"DPPA2\/4 and SUMO E3 ligase PIAS4 opposingly regulate zygotic transcriptional program","volume":"17","author":"Yan","year":"2019","journal-title":"PLoS Biol"},{"key":"10.1016\/j.ceb.2025.102579_sref67","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1038\/s41594-023-01038-z","article-title":"A genome-wide screen reveals new regulators of the 2-cell-like cell state","volume":"30","author":"Gupta","year":"2023","journal-title":"Nat Struct Mol Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib68","doi-asserted-by":"crossref","first-page":"C655","DOI":"10.1152\/ajpcell.00116.2019","article-title":"SMCHD1 terminates the first embryonic genome activation event in mouse two-cell embryos and contributes to a transcriptionally repressive state","volume":"317","author":"Ruebel","year":"2019","journal-title":"Am J Physiol Cell Physiol"},{"key":"10.1016\/j.ceb.2025.102579_bib69","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1038\/nsmb.3066","article-title":"Early embryonic-like cells are induced by downregulating replication-dependent chromatin assembly","volume":"22","author":"Ishiuchi","year":"2015","journal-title":"Nat Struct Mol Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib70","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1016\/j.stem.2018.10.001","article-title":"SUMO safeguards somatic and pluripotent cell identities by enforcing distinct chromatin states","volume":"23","author":"Cossec","year":"2018","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.ceb.2025.102579_bib71","doi-asserted-by":"crossref","DOI":"10.15252\/embr.201847382","article-title":"DPPA2 and DPPA4 are necessary to establish a 2C-like state in mouse embryonic stem cells","volume":"20","author":"De Iaco","year":"2019","journal-title":"EMBO Rep"},{"key":"10.1016\/j.ceb.2025.102579_bib72","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1101\/gad.321174.118","article-title":"Dppa2 and Dppa4 directly regulate the Dux-driven zygotic transcriptional program","volume":"33","author":"Eckersley-Maslin","year":"2019","journal-title":"Genes Dev"},{"key":"10.1016\/j.ceb.2025.102579_bib73","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/j.cell.2018.05.043","article-title":"A LINE1-Nucleolin partnership regulates early development and ESC identity","volume":"174","author":"Percharde","year":"2018","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2025.102579_bib74","doi-asserted-by":"crossref","first-page":"6365","DOI":"10.1038\/s41467-021-26576-2","article-title":"rRNA biogenesis regulates mouse 2C-like state by 3D structure reorganization of peri-nucleolar heterochromatin","volume":"12","author":"Yu","year":"2021","journal-title":"Nat Commun"},{"key":"10.1016\/j.ceb.2025.102579_bib75","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1101\/gad.349172.121","article-title":"Nucleolar-based Dux repression is essential for embryonic two-cell stage exit","volume":"36","author":"Xie","year":"2022","journal-title":"Genes Dev"},{"key":"10.1016\/j.ceb.2025.102579_bib76","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/s41556-019-0453-8","article-title":"Maternal factor NELFA drives a 2C-like state in mouse embryonic stem cells","volume":"22","author":"Hu","year":"2020","journal-title":"Nat Cell Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib77","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1038\/s42003-024-06507-9","article-title":"A CRISPR\/Cas9 screen in embryonic stem cells reveals that Mdm2 regulates totipotency exit","volume":"7","author":"Gao","year":"2024","journal-title":"Commun Biol"},{"key":"10.1016\/j.ceb.2025.102579_bib78","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1016\/j.stem.2022.01.014","article-title":"8C-like cells capture the human zygotic genome activation program in vitro","volume":"29","author":"Taubenschmid-Stowers","year":"2022","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.ceb.2025.102579_sref79","article-title":"LINE1 and PRC2 control nucleolar organization and repression of the 8C state in human ESCs","author":"Zhang","year":"2024","journal-title":"Dev Cell"},{"key":"10.1016\/j.ceb.2025.102579_bib80","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1038\/s41582-022-00762-2","article-title":"Facioscapulohumeral muscular dystrophy: the road to targeted therapies","volume":"19","author":"Tihaya","year":"2023","journal-title":"Nat Rev Neurol"}],"container-title":["Current Opinion in Cell Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0955067425001176?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0955067425001176?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,26]],"date-time":"2026-04-26T08:02:25Z","timestamp":1777190545000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0955067425001176"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10]]},"references-count":80,"alternative-id":["S0955067425001176"],"URL":"https:\/\/doi.org\/10.1016\/j.ceb.2025.102579","relation":{},"ISSN":["0955-0674"],"issn-type":[{"value":"0955-0674","type":"print"}],"subject":[],"published":{"date-parts":[[2025,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Epigenetic regulation of DUX4: From embryogenesis to muscular degeneration","name":"articletitle","label":"Article Title"},{"value":"Current Opinion in Cell Biology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ceb.2025.102579","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Authors. Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"102579"}}