诱导性多功能干细胞是诱导成体细胞重新编码生成的一类多潜能干细胞.诱导性多功能干细胞可以分化成具有心肌特异性分子标志、结构和功能的细胞.遗传性长QT综合征是一类与QT间期延长有关的遗传性心脏病.通过对长QT综合征家系患者的成体细胞进行诱导,生成诱导性多功能干细胞,并引导其分化成心肌样细胞,利用免疫组化、基因表达分析、全细胞膜片钳记录、微电极矩阵记录等方法对长QT综合征来源的诱导性多功能干细胞并引导其分化成心肌样细胞特征进行研究,对于阐明不同突变导致长QT综合征的发病机制、有效进行药物筛查和心脏毒性试验具有重要意义,并且为在细胞水平治疗长QT综合征提供实验依据.%Induced pluripotent stem cells ( iPS cells ) are a type of pluripoent stem cell artificially derived from the adult somatic cells by reprogramming the cells into a pluripotent state. iPS cells can differentiate into myocytes with cardiac-specific molecular, structural, and functional properties. These results, coupled with the potential of this technology to generate patient-specific iPS cell lines, hold great promise. Long QT syndromes are heritable disease associated with prolongation of the QT interval on an electrocardiogram. iPS cell cardiomyocytes ( iPS-CMs ) from patients with LQTS, which offer a potentially unlimited source of material for study. With the use of immunohistochemistry, gene analyze, whole cell patch clamp and micro-motor matrix record, cardiac like myocytes are being characterized to show the disease' s characteristics, establishing a convenient and powerful system to recapitulate complex physiological phenotypes, probe toxicological testing and drug screening, clarify the novel mechanistic insights and rectify gene defects at the cellular and molecular level.
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