CCDC141 is a multifunctional coiled-coil protein with critical roles in embryonic development, cardiac function, and neurological processes. During early development, CCDC141 regulates left-right axis formation by controlling cilia function in the Kupffer's vesicle through HDAC6-mediated α-tubulin acetylation 1. In the heart, CCDC141 functions as a mechanoadaptive factor by interacting with the sarcomeric connectin/titin filament system and nuclear envelope protein nesprin-1 at costameres 2. CCDC141 deletion impairs cardiomyocyte mitochondrial function and increases susceptibility to heart failure under pressure overload, with stress-induced nuclear relocalization suggesting a role in maintaining nuclear integrity 2. Genetically, CCDC141 variants associate with multiple cardiac arrhythmias including accessory atrioventricular pathways, supraventricular tachycardia, and sinus node dysfunction 345. Epistatic interactions between CCDC141, TTN, and IGF1R regulate cardiac hypertrophy 6. Beyond cardiology, CCDC141 mutations associate with laterality defects and restless legs syndrome in migraineurs, implicating roles in dopaminergic neural development 7. These findings position CCDC141 as a therapeutic target for cardiac mechanoadaptation and arrhythmia management.