{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T08:53:18Z","timestamp":1782463998575,"version":"3.54.5"},"reference-count":164,"publisher":"Wiley","license":[{"start":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T00:00:00Z","timestamp":1764806400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["physoc.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Physiology"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Inherited primary arrhythmia syndromes (IPAS) are genetic heart diseases associated with an elevated risk of sudden cardiac death, particularly in young individuals. Modelling these rare and serious conditions is essential to elucidate their mechanisms and to identify new treatments. Most genes involved in IPAS (e.g., congenital long\u2010QT syndrome, catecholaminergic polymorphic ventricular tachycardia, calcium\u2010release deficiency syndrome, Andersen\u2010Tawil syndrome, Timothy syndrome, calmodulinopathies, and short\u2010QT syndrome) are conserved in\n                    <jats:italic>Caenorhabditis elegans<\/jats:italic>\n                    , a model organism that offers powerful genetic tools for precise gene manipulation, including knock\u2010in, knock\u2010out, and knock\u2010down approaches.\n                    <jats:italic>In vivo<\/jats:italic>\n                    studies in\n                    <jats:italic>C. elegans<\/jats:italic>\n                    can be used to characterize the consequences of genetic variants (at molecular, cellular, tissue, and behavioural scales), to identify new regulatory proteins, and to perform drug testing. Here we summarize the characteristics of human IPAS and highlight the accumulating evidence that supports the utility of\n                    <jats:italic>C. elegans<\/jats:italic>\n                    as a simple yet powerful\n                    <jats:italic>in vivo<\/jats:italic>\n                    model for these diseases, capable of filling the gap between\n                    <jats:italic>in vitro<\/jats:italic>\n                    studies and complex transgenic animal models.\n                    <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                      <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/tjp70252-gra-0001-m.png\">\n                        <jats:alt-text>image<\/jats:alt-text>\n                      <\/jats:graphic>\n                    <\/jats:boxed-text>\n                  <\/jats:p>","DOI":"10.1113\/jp289661","type":"journal-article","created":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T11:08:52Z","timestamp":1764846532000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["<i>Caenorhabditis elegans<\/i>\n                    as an\n                    <i>in vivo<\/i>\n                    model system for human inherited primary arrhythmia syndromes"],"prefix":"10.1113","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3742-9674","authenticated-orcid":false,"given":"Antoine","family":"Delini\u00e8re","sequence":"first","affiliation":[{"name":"MeLiS Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5284, INSERM U1314  Lyon France"},{"name":"Centre de r\u00e9f\u00e9rence national des troubles du rythme cardiaque h\u00e9r\u00e9ditaires ou rares de Lyon (CERA) Institut de Cardiologie, H\u00f4pital Louis Pradel, Hospices Civils de Lyon  Bron France"},{"name":"Service de Rythmologie Institut de Cardiologie, H\u00f4pital Louis Pradel, Hospices Civils de Lyon  Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1734-1915","authenticated-orcid":false,"given":"Thomas","family":"Boulin","sequence":"additional","affiliation":[{"name":"MeLiS Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5284, INSERM U1314  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ma\u00eblle","family":"Jospin","sequence":"additional","affiliation":[{"name":"MeLiS Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5284, INSERM U1314  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Janin","sequence":"additional","affiliation":[{"name":"Laboratoire de cardiog\u00e9n\u00e9tique mol\u00e9culaire Centre de biologie et pathologie Est, Hospices Civils de Lyon  Bron France"},{"name":"PGNM Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5261, INSERM U1315  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilles","family":"Millat","sequence":"additional","affiliation":[{"name":"Laboratoire de cardiog\u00e9n\u00e9tique mol\u00e9culaire Centre de biologie et pathologie Est, Hospices Civils de Lyon  Bron France"},{"name":"PGNM Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5261, INSERM U1315  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Chevalier","sequence":"additional","affiliation":[{"name":"MeLiS Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5284, INSERM U1314  Lyon France"},{"name":"Centre de r\u00e9f\u00e9rence national des troubles du rythme cardiaque h\u00e9r\u00e9ditaires ou rares de Lyon (CERA) Institut de Cardiologie, H\u00f4pital Louis Pradel, Hospices Civils de Lyon  Bron France"},{"name":"Service de Rythmologie Institut de Cardiologie, H\u00f4pital Louis Pradel, Hospices Civils de Lyon  Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7659-4771","authenticated-orcid":false,"given":"Olga","family":"Andrini","sequence":"additional","affiliation":[{"name":"MeLiS Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5284, INSERM U1314  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,4]]},"reference":[{"issue":"1","key":"e_1_2_2_2_1","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1016\/j.hrthm.2016.09.012","article-title":"Beta\u2010blocker therapy for long QT syndrome and catecholaminergic polymorphic ventricular tachycardia: Are all beta\u2010blockers equivalent?","volume":"14","author":"Ackerman M. 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