Generation of iPSC line from patient with arrhythmogenic right ventricular cardiomyopathy carrying mutations in PKP2 gene


Human iPSC line was generated from patient-specific adipose tissue-derived mesenchymal multipotent stromal cells carrying two mutations in plakophilin-2 (PKP2) gene using non-integrative reprogramming method. Reprogramming factors OCT4, KLF4, SOX2, CMYC were delivered using Sendai viruses. Pluripotency was confirmed in vitro using immunofluorescence and RT-PCR analysis and in vivo by teratoma assay. The reported iPSC line could be useful tool for in vitro modeling of arrhythmogenic right ventricular cardiomyopathy. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

Authors Khudiakov A, Kostina D, Zlotina A, Yany N, Sergushichev A, Pervunina T, Tomilin A, Kostareva A, Malashicheva A
Journal Stem cell research
Publication Date 2017 Oct;24:85-88
PubMed 29034900
DOI 10.1016/j.scr.2017.08.014

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