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This review explores the molecular architecture and multifunctional roles of CASQ2, beginning with its domain organization and Ca\n                    <jats:sup>2+<\/jats:sup>\n                    -binding properties and detecting how its folding and supramolecular assembly modulate Ca\n                    <jats:sup>2+<\/jats:sup>\n                    storage and release within cardiac muscle. Post-translational modifications, genetic regulatory mechanisms and CASQ2\u2019s multipartner interactome; including Ryanodine receptor 2 (RyR2), triadin and junctin are also discussed to highlight potential models in which complex stoichiometry and luminal Ca\n                    <jats:sup>2+<\/jats:sup>\n                    dictate channel refractoriness and excitation-contraction coupling. Disruption of CASQ2 function is increasingly recognized as a driver of certain types of arrhythmias, notably catecholaminergic polymorphic ventricular tachycardia (CPVT) and heightened risk of sudden cardiac death. This review appraises contemporary therapies that focus on pharmacological and device-based interventions and surveys next-generation strategies that aim to directly stabilize CASQ2 or target its gene expression. Despite therapeutic advances, the challenges remain; and a translational agenda aligning mechanism with therapy is proposed. By integrating recent structural, functional, regulatory and pathological insights, this review provides a conceptual framework for the pivotal role of CASQ2 in arrhythmogenesis and positions CASQ2 biology at the center of precision cardiology.\n                  <\/jats:p>","DOI":"10.1007\/s13105-026-01137-7","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T01:48:13Z","timestamp":1769996893000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["The emerging role of calsequestrin 2: from calcium sensor and modulator to arrhythmia driver"],"prefix":"10.1007","volume":"82","author":[{"given":"Humam Emad","family":"Rajha","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baha H.","family":"Abuajameia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali Mohamed","family":"Barhoma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim El-Arabi","family":"Hashem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zeyaul","family":"Islam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Lai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. 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