Modeling hypertrophic cardiomyopathy with human cardiomyocytes derived from induced pluripotent stem cells

One of the obstacles in studying the pathogenesis of hypertrophic cardiomyopathy (HCM) is the poor availability of myocardial tissue samples at the early stages of disease development. This has been addressed by the advent of induced pluripotent stem cells (iPSCs), which allow us to differentiate pa...

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Bibliographic Details
Published in:Stem cell research & therapy 2022-06, Vol.13 (1), p.232-232, Article 232
Main Authors: Li, Jiangtao, Feng, Xin, Wei, Xiang
Format: Article
Language:English
Subjects:
Adenoviruses
Analysis
Calcium (mitochondrial)
Cardiomyocytes
Cardiomyopathy
Cardiomyopathy, Hypertrophic
Cell culture
Cell differentiation
Contractility
Developmental stages
Disease
Disease modeling
Drug development
Efficiency
Electrical stimuli
Fibroblasts
Genes
Genome editing
Genomes
Genomics
Genotype & phenotype
Heart
Heart cells
Hypertrophic cardiomyopathy
Induced pluripotent stem cells
iPSC-derived cardiomyocytes
Mitochondria
Mutation
Pathogenesis
Patients
Pluripotency
Proteins
Review
Stem cells
Structure-function relationships
Viruses
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Summary:One of the obstacles in studying the pathogenesis of hypertrophic cardiomyopathy (HCM) is the poor availability of myocardial tissue samples at the early stages of disease development. This has been addressed by the advent of induced pluripotent stem cells (iPSCs), which allow us to differentiate patient-derived iPSCs into cardiomyocytes (iPSC-CMs) in vitro. In this review, we summarize different approaches to establishing iPSC models and the application of genome editing techniques in iPSC. Because iPSC-CMs cultured at the present stage are immature in structure and function, researchers have attempted several methods to mature iPSC-CMs, such as prolonged culture duration, and mechanical and electrical stimulation. Currently, many researchers have established iPSC-CM models of HCM and employed diverse methods for performing measurements of cellular morphology, contractility, electrophysiological property, calcium handling, mitochondrial function, and metabolism. Here, we review published results in humans to date within the growing field of iPSC-CM models of HCM. Although there is no unified consensus, preliminary results suggest that this approach to modeling disease would provide important insights into our understanding of HCM pathogenesis and facilitate drug development and safety testing.
ISSN:1757-6512
1757-6512
DOI:10.1186/s13287-022-02905-0