Human iPSC cell lines (FAMRCi004-A and FAMRCi004-B) were generated from patient with progressive cardiac conduction disease and sick sinus syndrome carrying DSP p.His1684Arg genetic variant. Patient-specific adipose tissue-derived mesenchymal multipotent stromal cells were reprogrammed using non-integrative Sendai viruses. Established iPSC lines showed normal karyotype, expressed pluripotent markers and were able to differentiate toward three germ layers in vitro. The reported iPSC lines could be useful tool for in vitro modeling of progressive cardiac conduction disease associated with mutations in desmosomal genes.

中文翻译：

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从携带遗传变异DSP p.His1684Arg的家族性进行性心脏传导障碍的患者中产生两条iPSC系（FAMRCi004-A和FAMRCi004-B）。

人类iPSC细胞系（FAMRCi004-A和FAMRCi004-B）是由患有进行性心脏传导疾病和病态窦房结综合征的患者产生的，带有DSP p.His1684Arg基因变异。使用非整合型仙台病毒对患者特定的脂肪组织来源的间充质多能基质细胞进行重新编程。建立的iPSC品系显示出正常的核型，表达多能性标记，并能够在体外分化为三个胚层。报道的iPSC品系可能是有用的工具，可用于体外建模与桥粒基因突变相关的进行性心脏传导疾病。