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

Abstract:

: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.

journal_name

Stem Cell Res

journal_title

Stem cell research

authors

Khudiakov A,Kostina D,Zlotina A,Yany N,Sergushichev A,Pervunina T,Tomilin A,Kostareva A,Malashicheva A

doi

10.1016/j.scr.2017.08.014

subject

Has Abstract

pub_date

2017-10-01 00:00:00

pages

85-88

eissn

1873-5061

issn

1876-7753

pii

S1873-5061(17)30177-0

journal_volume

24

pub_type

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