Cardiomyopathy is a pathological condition characterized by cardiac pump failure due to myocardial dysfunction and the major cause of advanced heart failure requiring heart transplantation. Although optimized medical therapies have been developed for heart failure during the last few decades, some patients with cardiomyopathy exhibit advanced heart failure and are refractory to medical therapies. Desmosome, which is a dynamic cell-to-cell junctional component, maintains the structural integrity of heart tissues. Genetic mutations in desmo
Core Tip: Prevention of advanced heart failure caused by cardiomyopathy is an urgent unmet need in the field of cardiovascular medicine. Desmosome, a cell-to-cell junctional component, maintains the structural integrity of heart tissues. Genetic mutations in desmosomal genes cause desmosome-related cardiomyopathy, an intractable disease refractory to standard medical therapies. This review introduces the recent advances in disease modeling of desmosome-related cardiomyopathy caused by PKP2 mutations using induced pluripotent stem cell-derived cardiomyocytes.
- Citation: Higo S. Disease modeling of desmosome-related cardiomyopathy using induced pluripotent stem cell-derived cardiomyocytes. World J Stem Cells 2023; 15(3): 71-82
- URL: https://www.wjgnet.com/1948-0210/full/v15/i3/71.htm
- DOI: https://dx.doi.org/10.4252/wjsc.v15.i3.71