SNTA1 encodes α-1-syntrophin, an adapter protein that organizes subcellular localization of membrane proteins and links receptors to the actin cytoskeleton via the dystrophin glycoprotein complex. In cardiomyocytes, SNTA1 plays a critical role in proper localization of Nav1.5 sodium channels and Kir2.1 potassium channels, which are essential for cardiac excitability and conduction 1. SNTA1 deficiency disrupts cardiac calcium homeostasis, resulting in hypertrophic phenotype, reduced contractility, and impaired calcium transient intensity in human cardiomyocytes 2. Additionally, SNTA1 maintains perivascular aquaporin-4 localization in the brain; its loss impairs glymphatic clearance and promotes amyloid-β accumulation, potentially contributing to Alzheimer's disease pathology 3. SNTA1 mutations are associated with cardiac arrhythmias including Long QT syndrome, Brugada syndrome, and sudden infant death syndrome 1. However, evidence for SNTA1 as a definitive Long QT syndrome causative gene is limited; an international evidence-based reappraisal classified SNTA1 among genes with only limited or disputed evidence for LQTS causation 4. Outside the cardiovascular system, SNTA1 regulates Rac1 activation and reactive oxygen species generation in breast cancer cells, influencing cell migration and malignant potential 5.