{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T04:49:33Z","timestamp":1784609373694,"version":"3.55.0"},"reference-count":43,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2021,6,16]],"date-time":"2021-06-16T00:00:00Z","timestamp":1623801600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Instituto de Salud Carlos III and FEDER Union Europea"},{"name":"Una forma de hacer Europa","award":["PI18\/01231"],"award-info":[{"award-number":["PI18\/01231"]}]},{"name":"Una forma de hacer Europa","award":["PI18\/01582"],"award-info":[{"award-number":["PI18\/01582"]}]},{"name":"Una forma de hacer Europa","award":["PI18\/01231"],"award-info":[{"award-number":["PI18\/01231"]}]},{"name":"FEDER Union Europea"},{"name":"Una forma de hacer Europa","award":["PI18\/01582"],"award-info":[{"award-number":["PI18\/01582"]}]},{"name":"La Fe Biobank","award":["PT17\/0015\/0043"],"award-info":[{"award-number":["PT17\/0015\/0043"]}]},{"name":"Memorial Nacho Barber\u00e1. 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ACM is mainly caused by mutations in genes encoding desmosome proteins. Desmosomes are cell\u2013cell adhesion structures and hubs for mechanosensing and mechanotransduction. The objective was to identify the dysregulated molecular and biological pathways in human ACM in the absence of overt heart failure.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Transcriptomes in the right ventricular endomyocardial biopsy samples from three independent individuals carrying truncating mutations in the DSP gene and five control samples were analysed by RNA-Seq (discovery group). These cases presented with cardiac arrhythmias and had a normal right ventricular function. The RNA-Seq analysis identified \u223c5000 differentially expressed genes (DEGs), which predicted suppression of the Hippo and canonical WNT pathways, among others. Dysregulated genes and pathways, identified by RNA-Seq, were tested for validation in the right and left ventricular tissues from five independent autopsy-confirmed ACM cases with defined mutations (validation group), who were victims of SCD and had no history of heart failure. Protein levels and nuclear localization of the cWNT and Hippo pathway transcriptional regulators were reduced in the right and left ventricular validation samples. In contrast, levels of acetyltransferase EP300, known to suppress the Hippo and canonical WNT pathways, were increased and its bona fide target TP53 was acetylated. RNA-Seq data identified apical junction, reflective of cell\u2013cell attachment, as the most disrupted biological pathway, which were corroborated by disrupted desmosomes and intermediate filament structures. Moreover, the DEGs also predicted dysregulation of over a dozen canonical signal transduction pathways, including the Tec kinase and integrin signalling pathways. The changes were associated with increased apoptosis and fibro-adipogenesis in the ACM hearts.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion\u2003<\/jats:title>\n                  <jats:p>Altered apical junction structures are associated with activation of the EP300-TP53 and suppression of the Hippo\/cWNT pathways in human ACM caused by defined mutations in the absence of an overt heart failure. The findings implicate altered mechanotransduction in the pathogenesis of ACM.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/cvr\/cvab197","type":"journal-article","created":{"date-parts":[[2021,6,14]],"date-time":"2021-06-14T19:11:21Z","timestamp":1623697881000},"page":"1466-1478","source":"Crossref","is-referenced-by-count":43,"title":["The EP300\/TP53 pathway, a suppressor of the Hippo and canonical WNT pathways, is activated in human hearts with arrhythmogenic cardiomyopathy in the absence of overt heart failure"],"prefix":"10.1093","volume":"118","author":[{"given":"Leila","family":"Rouhi","sequence":"first","affiliation":[{"name":"Department of Medicine, Center for Cardiovascular Genetics, Institute of Molecular Medicine, University of Texas Health Sciences Center at Houston, 6770 Bertner Ave, Suite C900A, Houston, TX 77030, 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