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