LMNA mutations are often linked to laminopathies characterized by tissue-specific disorders. We generated two induced pluripotent stem cells lines from patient carrying genetic variant LMNA p.Asp357Val associated with paroxysmal ventricular tachycardia and myopathy. Reprogramming of patient's peripheral blood mononuclear cells was performed using Sendai viruses. Characterization of the FAMRCi005-A and FAMRCi005-B lines revealed that generated iPSC lines expressed pluripotent stem cell markers, had normal karyotype and demonstrated triliniage differentiation ability. Generated cell lines can be used to investigate the molecular links between LMNA genetic variants and cardiac disorders.

LMNA突变通常与以组织特异性疾病为特征的椎间盘突出症相关。我们从携带遗传变异LMNA p.Asp357Val的患者中产生了两种与阵发性室性心动过速和肌病相关的诱导性多能干细胞系。使用仙台病毒对患者的外周血单核细胞进行重编程。FAMRCi005-A和FAMRCi005-B系的鉴定表明，生成的iPSC系表达了多能干细胞标记，具有正常的核型，并显示了三向分化能力。生成的细胞系可用于研究LMNA遗传变异与心脏疾病之间的分子联系。