Uncovering Inherited Cardiomyopathy With Human Induced Pluripotent Stem Cells.

Uncovering Inherited Cardiomyopathy With Human Induced Pluripotent Stem Cells.
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利用人类诱导多能干细胞揭示遗传性心肌病

DOI:
10.3389/fcell.2021.672039
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发表时间:
2021
影响因子:
5.5
通讯作者:
Hu S
Hu S
中科院分区:
生物学2区
文献类型:
--
作者:
Jiang X;Chen Y;Liu X;Ye L;Yu M;Shen Z;Lei W;Hu S

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在过去的几十年里,研究人员发现了遗传缺陷对原发性心肌病发病机制的贡献,并试图通过建立多种疾病模型来解释这些疾病的发病机制。尽管人类心脏组织和原代心肌细胞在模拟人类心脏病方面具有优势,但它们难以在体外获得和培养。转基因动物模型中产生的缺陷在分子水平上与人类疾病明显不同。人类诱导多能干细胞 (hiPSC) 的出现提供了前所未有的机会,可以利用患者特异性 hiPSC 衍生的心肌细胞在体外进一步研究遗传性心肌病的致病机制。在这篇综述中,我们将总结使用 hiPSC 进行体外遗传性心肌病建模的最新进展。
In the past decades, researchers discovered the contribution of genetic defects to the pathogenesis of primary cardiomyopathy and tried to explain the pathogenesis of these diseases by establishing a variety of disease models. Although human heart tissues and primary cardiomyocytes have advantages in modeling human heart diseases, they are difficult to obtain and culture in vitro. Defects developed in genetically modified animal models are notably different from human diseases at the molecular level. The advent of human induced pluripotent stem cells (hiPSCs) provides an unprecedented opportunity to further investigate the pathogenic mechanisms of inherited cardiomyopathies in vitro using patient-specific hiPSC-derived cardiomyocytes. In this review, we will make a summary of recent advances in in vitro inherited cardiomyopathy modeling using hiPSCs.
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