Abstract
Cardiovascular diseases (CVDs) are a class of disorders affecting the heart or blood vessels. Despite progress in clinical research and therapy, CVDs still represent the leading cause of mortality and morbidity worldwide. The hallmarks of cardiac diseases include heart dysfunction and cardiomyocyte death, inflammation, fibrosis, scar tissue, hyperplasia, hypertrophy, and abnormal ventricular remodeling. The loss of cardiomyocytes is an irreversible process that leads to fibrosis and scar formation, which, in turn, induce heart failure with progressive and dramatic consequences. Both genetic and environmental factors pathologically contribute to the development of CVDs, but the precise causes that trigger cardiac diseases and their progression are still largely unknown. The lack of reliable human model systems for such diseases has hampered the unraveling of the underlying molecular mechanisms and cellular processes involved in heart diseases at their initial stage and during their progression. Over the past decade, significant scientific advances in the field of stem cell biology have literally revolutionized the study of human disease in vitro. Remarkably, the possibility to generate disease-relevant cell types from induced pluripotent stem cells (iPSCs) has developed into an unprecedented and powerful opportunity to achieve the long-standing ambition to investigate human diseases at a cellular level, uncovering their molecular mechanisms, and finally to translate bench discoveries into potential new therapeutic strategies. This review provides an update on previous and current research in the field of iPSC-driven cardiovascular disease modeling, with the aim of underlining the potential of stem-cell biology-based approaches in the elucidation of the pathophysiology of these life-threatening diseases.
Highlights
Cardiovascular diseases (CVDs) are the leading cause of mortality and morbidity worldwide and the development of novel therapeutic treatments still remains a major research goal
Patient-specific induced pluripotent stem cells (iPSCs) were differentiated into cardiomyocytes that were shown to be larger, with a highly structured and organized sarcomere and a preferential nuclear localization of NFATc4, which is responsible for the hypertrophic phenotype seen in LEOPARD syndrome (LS) patient’s CMs compared to their healthy counterpart [124]
A comprehensive understanding of the molecular mechanisms underlying human cardiac diseases has been hampered by the lack of reliable model systems that mirror the human disease phenotype
Summary
Cardiovascular diseases (CVDs) are the leading cause of mortality and morbidity worldwide and the development of novel therapeutic treatments still remains a major research goal. Human pluripotent stem cells (hPSCs), including embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs), have the capability to self-renew indefinitely and to differentiate into derivatives of the three germ layers (ectoderm, mesoderm, and endoderm) These features make them very promising in a variety of basic research and clinical applications such as developmental biology [5], drug screening, disease modelling, and regenerative medicine. IPSCs derived from healthy individuals or diseased patients carry the genome of their cell of origin and can be differentiated into any cell type, including cells not otherwise accessible, representing a powerful cell-based model system for human diseases, genetic investigations, drug screening, and personalized therapy [11].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
