Modelling Human Channelopathies Using Induced Pluripotent Stem Cells: A Comprehensive Review
Figure 2
(a) Schematic presentation of iPSC-derived cardiomyocytes’ retrieval. Based on easy accessible donor cells, like, for example, fibroblasts and consequent overexpression of different pluripotency factors, reprogrammed donor cells fall into a state of pluripotency (iPS-Cells). By various forms of directed cardiac differentiation, cardiomyocyte-like cells show essential characteristics of adult cardiomyocytes, maintaining their LQT-/non-LQT-phenotype. (b) Possible applications for iPSC-derived cardiomyocytes: (i) disease modelling for better understanding of genetic and epigenetic causation of LQTS; (ii) large-scale drug screening for both patient-specific and nonspecific pharmacotherapy; (iii) circumventing transplantation-associated immunogenicity by patient-specific cell therapy.