TY - JOUR
T1 - Cardiomyopathy in a Dish
T2 - Using Human Inducible Pluripotent Stem Cells to Model Inherited Cardiomyopathies
AU - Kamdar, Forum
AU - Klaassen Kamdar, Andre
AU - Koyano-Nakagawa, Naoko
AU - Garry, Mary G.
AU - Garry, Daniel J.
N1 - 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.
PY - 2015/9/1
Y1 - 2015/9/1
N2 - 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.
AB - 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.
KW - Cardiomyopathy
KW - disease models
KW - stem cells
UR - http://www.scopus.com/inward/record.url?scp=84940614291&partnerID=8YFLogxK
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U2 - 10.1016/j.cardfail.2015.04.010
DO - 10.1016/j.cardfail.2015.04.010
M3 - Review article
C2 - 25934595
AN - SCOPUS:84940614291
SN - 1071-9164
VL - 21
SP - 761
EP - 770
JO - Journal of Cardiac Failure
JF - Journal of Cardiac Failure
IS - 9
ER -
