Modeling of inherited cardiomyopathies based on human differentiated induced pluripotent stem cells derivatives

One of the obstacles on the way to a better understanding of the pathogenesis of cardiomyopathies has been unavailability of tissue samples from the heart in the early stages of the disease. This has been changed with the emergence of patient-specific induced pluripotent stem cells (iPSC), from which cardiomyocytes in vitro can be derived. The feature of iPSC technology is that given the effects of thousands of individual gene variants, a phenotype of differentiated derivatives of these cells will provide more information about a specific disease of the patient than by simple genotyping. This article summarizes what is known about the models of cardiomyopathies in vitro, with an emphasis on identifying the molecular and cellular pathogenesis mechanisms, treatment and testing of new drugs.