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Generation of patient-specific induced pluripotent stem cell-derived cardiomyocytes as a cellular model of arrhythmogenic right ventricular cardiomyopathy
Author(s) -
Dongrui Ma,
Heming Wei,
Jun Lü,
Shuswen Ho,
Guangqing Zhang,
Xiaoming Sun,
Yingzi Oh,
Suat Hoon Tan,
Mah Lee Ng,
Winston Shim,
Philip Wong,
Reginald Liew
Publication year - 2012
Publication title -
european heart journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.336
H-Index - 293
eISSN - 1522-9645
pISSN - 0195-668X
DOI - 10.1093/eurheartj/ehs226
Subject(s) - plakoglobin , induced pluripotent stem cell , desmoplakin , intercalated disc , medicine , connexin , microbiology and biotechnology , myocyte , cardiomyopathy , reprogramming , pathology , cell , gap junction , biology , signal transduction , heart failure , embryonic stem cell , gene , catenin , genetics , wnt signaling pathway , intracellular
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary heart muscle disorder associated with sudden cardiac death. Its pathophysiology is still poorly understood. We aimed to produce an in vitro cellular model of ARVC using patient-specific induced pluripotent stem cell (iPSC)-derived cardiomyocytes and determine whether the model could recapitulate key features of the disease phenotype.

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