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

Aleksandr Khudiakov | Daria Kostina | Anna Zlotina | Natalia Yany | Alexey Sergushichev | Tatiana Pervunina | Alexey Tomilin | Anna Kostareva | Anna Malashicheva
Stem cell research | 2017

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.

Pubmed ID: 29034900

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