Cardiomyopathy in a Dish: Using Human Inducible Pluripotent Stem Cells to Model Inherited Cardiomyopathies

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Abstract

Inherited cardiomyopathies, including hypertrophic cardiomyopathy, dilated cardiomyopathies, arrythmogenic right ventricular cardiomyopathy, and other inherited forms of heart failure, represent a unique set of genetically defined cardiovascular disease processes. Unraveling the molecular mechanisms of these deadly forms of human heart disease has been challenging, but recent groundbreaking scientific advances in stem cell technology have allowed for the generation of patient-specific human inducible stem cell (hiPSC)-derived cardiomyocytes (CMs). hiPSC-derived CMs retain the genetic blueprint of the patient, they can be maintained in culture, and they recapitulate the phenotypic characteristics of the disease in vitro, thus serving as a disease in a dish. This review provides an overview of in vitro modeling of inherited cardiomyopathies with the use of patient-specific hiPSC-derived CMs.

Original languageEnglish (US)
Pages (from-to)761-770
Number of pages10
JournalJournal of cardiac failure
Volume21
Issue number9
DOIs
StatePublished - Sep 1 2015

Bibliographical note

Funding Information:
The authors are grateful for artwork assistance by Ms Cynthia DeKay. Funding is acknowledged from the NIH ( R01HL122576 and U01HL100407).

Publisher Copyright:
© 2015 Published by Elsevier Inc.

Keywords

  • Cardiomyopathy
  • disease models
  • stem cells

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