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Variants (pathogenic) of the <i>LMNA</i> gene are a common cause of familial dilated cardiomyopathy (DCM), which is characterised by early-onset atrioventricular (AV) block, atrial fibrillation and ventricular tachyarrhythmias (VTs), and progressive heart failure. The unstable internal nuclear lamina observed in <i>LMNA</i>-related DCM is a consequence of the disassembly of lamins A and C. This suggests that <i>LMNA</i> variants produce truncated or alternative forms of protein that alter the nuclear structure and the signalling pathway related to cardiac muscle diseases. To date, the pathogenic mechanisms and phenotypes of <i>LMNA</i>-related DCM have been studied using different platforms, such as patient-specific induced pluripotent stem-cell-derived cardiomyocytes (iPSC-CMs) and transgenic mice. In this review, point variants in the <i>LMNA</i> gene that cause autosomal dominantly inherited forms of <i>LMNA</i>-related DCM are summarised. In addition, potential therapeutic targets based on preclinical studies of <i>LMNA</i> variants using transgenic mice and human iPSC-CMs are discussed. They include mitochondria deficiency, variants in nuclear deformation, chromatin remodelling, altered platelet-derived growth factor and ERK1/2-related pathways, and abnormal calcium handling.