THE ROLE OF DESMOPLAKIN IN CARDIAC DEVELOPMENT AND DISEASE

这个子项目是众多研究子项目之一

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Desmosomes are major cell adhesion junctions between cells that anchor the cell membrane to the intermediate filament network, and found particularly in tissues undergoing constant stress, such as the heart. Desmoplakin, is a central component of the desmosomal complex, which is highly expressed in the cardiac desmosomes. Although desmoplakin mutations have been associated with human right ventricular cardiomyopathies, such as arrhythmogenic right ventricle dysplasia (ARVD), its role in the heart and ARVD, remain unknown. To understand the structural and functional role of desmoplakin in the heart, we have generated cardiac-specific desmoplakin conditional knockout mice. To determine whether there are structural alterations in these knockout mice, we would like to perform TEM and immunolabeling studies.