Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes Afford New Opportunities in Inherited Cardiovascular Disease Modeling

Abstract

Fundamental studies of molecular and cellular mechanisms of cardiovascular disease pathogenesis are required to create more effective and safer methods of their therapy. The studies can be carried out only when model systems that fully recapitulate pathological phenotype seen in patients are used. Application of laboratory animals for cardiovascular disease modeling is limited because of physiological differences with humans. Since discovery of induced pluripotency generating induced pluripotent stem cells has become a breakthrough technology in human disease modeling. In this review, we discuss a progress that has been made in modeling inherited arrhythmias and cardiomyopathies, studying molecular mechanisms of the diseases, and searching for and testing drug compounds using patient-specific induced pluripotent stem cell-derived cardiomyocytes.

Figure 1

Induced pluripotent stem cell-based approach for human disease modeling. hiPSCs can be generated from human somatic cells using viruses, plasmids, modified RNA, and recombinant proteins. iPSCs are differentiated into various cell types for disease modeling, drug screening, and cell therapy.

Figure 2

Genome editing in cardiovascular disease modeling with induced pluripotent stem cells. Patient-specific induced pluripotent stem cells are corrected using genome editing tools to create a panel of isogenic iPSC lines that are differentiated into cardiomyocytes for disease modeling, drug discovery and screening, and cell therapy.