Given that the DDR is a biologically important process that ensures the maintenance of cellular homeostasis and prevents human disease, our study sheds light on a potential mechanism that may be deregulated in TOR1AIP1-associated disorders, such as cardiomyopathy and muscular dystrophy, and encourages a more detailed investigation of the physiological functions of this essential INM protein, including the effects of its deficiency on DDR dynamics. Here, TOR1AIP1 is linked to muscular dystrophy.