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Generation of Ventricular-Like HiPSC-Derived Cardiomyocytes and High-Quality Cell Preparations for Calcium Handling Characterization
Published on: January 17, 2020
Human-Induced Pluripotent Stem Cell-Based Modeling of Cardiac Storage Disorders
Bradley C Nelson1, Sherin I Hashem1, Eric D Adler2
1Department of Medicine, Division of Cardiology, University of California San Diego, 9500 Gilman Drive, Biomedical Research Facility, Room 1217 AA, La Jolla, CA, 92093, USA.
Purpose Of Review:
The aim of this study is to review the published human-induced pluripotent stem cell-derived cardiomyocyte (hiPSC-CM) models of cardiac storage disorders and to evaluate the limitations and future applications of this technology.
Recent Findings:
Several cardiac storage disorders (CSDs) have been modeled using patient-specific hiPSC-CMs, including Anderson-Fabry disease, Danon disease, and Pompe disease. These models have shown that patient-specific hiPSC-CMs faithfully recapitulate key phenotypic features of CSDs and respond predictably to pharmacologic manipulation. hiPSC-CMs generated from patients with CSDs are representative models of the patient disease state and can be used as an in vitro system for the study of human cardiomyocytes. While these models suffer from several limitations, they are likely to play an important role in future mechanistic studies of cardiac storage disorders and the development of targeted therapeutics for these diseases.
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