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This review comprehensively summarizes the clinical features and genetic traits of JLNS. The pathogenicity of the amino acid substitutions in Kv7.1 and mink was assessed using AlphaFold. Furthermore, we systematically evaluated recent progress in experimental models of JLNS as well as phenotypic characterization and mechanistic studies across these experimental platforms, encompassing nonexcitable cell systems, genetically engineered murine models, and patient\u2010specific induced pluripotent cell\u2010derived cardiomyocytes and inner ear hair cells.\n                  <\/jats:p>","DOI":"10.1002\/pdi3.70047","type":"journal-article","created":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T06:00:25Z","timestamp":1775714425000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Jervell and Lange\u2010Nielsen Syndrome Related Clinical Genetics and Experimental Models"],"prefix":"10.1002","volume":"4","author":[{"given":"Yafei","family":"Zhou","sequence":"first","affiliation":[{"name":"National Regional Children's Medical Center (Northwest), Key Laboratory of Precision Medicine to Pediatric Diseases of Shaanxi Province, Xi'an Key Laboratory of Children's Health and Diseases Shaanxi Institute for Pediatric Diseases Xi'an Children's Hospital Affiliated Children's Hospital of Xi'an Jiaotong University  Xi'an, Shaanxi China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher L.\u2010H.","family":"Huang","sequence":"additional","affiliation":[{"name":"Physiological Laboratory and Department of Biochemistry University of Cambridge  Cambridge UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8305-1978","authenticated-orcid":false,"given":"Ying","family":"Yang","sequence":"additional","affiliation":[{"name":"National Regional Children's Medical Center (Northwest), Key Laboratory of Precision Medicine to Pediatric Diseases of Shaanxi Province, Xi'an Key Laboratory of Children's Health and Diseases Shaanxi Institute for Pediatric Diseases Xi'an Children's Hospital Affiliated Children's Hospital of Xi'an Jiaotong 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