Abstract
Purpose of review:
This article provides an overview of the latest advances in in-vitro modeling of inherited cardiomyopathies using human-induced pluripotent stem cells (iPSCs).
Recent findings:
Inherited cardiomyopathies have been recently modeled by generating iPSCs from patients harboring mutations in genes associated with the pathogenesis of hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy/dysplasia.
Summary:
Patient-specific iPSCs and their differentiated cardiomyocytes (induced pluripotent stem cell-derived cardiomyocytes) now provide a novel model to study the underlying molecular mechanism of the pathogenesis of familial cardiomyopathies as well as for in-vitro drug screening and drug discovery.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Arrhythmogenic Right Ventricular Dysplasia* / drug therapy
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Arrhythmogenic Right Ventricular Dysplasia* / etiology
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Arrhythmogenic Right Ventricular Dysplasia* / pathology
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Cardiomyopathy, Dilated* / congenital
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Cardiomyopathy, Dilated* / drug therapy
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Cardiomyopathy, Dilated* / pathology
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Cardiomyopathy, Hypertrophic, Familial* / drug therapy
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Cardiomyopathy, Hypertrophic, Familial* / etiology
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Cardiomyopathy, Hypertrophic, Familial* / pathology
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Drug Discovery / methods
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Drug Discovery / trends
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Genome-Wide Association Study
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Humans
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In Vitro Techniques / methods
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In Vitro Techniques / trends
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Induced Pluripotent Stem Cells* / drug effects
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Induced Pluripotent Stem Cells* / pathology
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Models, Cardiovascular
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Myocytes, Cardiac* / drug effects
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Myocytes, Cardiac* / pathology