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Significance Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease that is selective to the right side of the heart and results in heart failure and sudden death. Genetic defects in desmosome components account for approximately 50% of human ARVC cases; in the other 50% of patients, however, the causes remain unknown. We show that inhibitor of apoptosis-stimulating protein of p53 (iASPP) is an important regulator of desmosomes. It interacts with desmoplakin and desmin in cardiomyocytes and regulates desmosome integrity and intermediate filaments. iASPP-deficient mice display pathological features of ARVC and die of sudden death. In human ARVC patients, cardiomyocytes exhibit reduced levels of iASPP at the cell junctions, suggesting that iASPP may be critical in ARVC pathogenesis.

iASPP, a previously unidentified regulator of desmosomes, prevents arrhythmogenic right ventricular cardiomyopathy (ARVC)-induced sudden death