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用于探索家族性心房颤动的PITX2缺陷型人诱导多能干细胞系的生成及心脏亚型特异性分化。

Generation and cardiac subtype-specific differentiation of PITX2-deficient human iPS cell lines for exploring familial atrial fibrillation.

作者信息

Marczenke Maike, Fell Jakob, Piccini Ilaria, Röpke Albrecht, Seebohm Guiscard, Greber Boris

机构信息

Human Stem Cell Pluripotency Laboratory, Max Planck Institute for Molecular Biomedicine, Münster, Germany; Chemical Genomics Centre of the Max Planck Society, Dortmund, Germany.

Human Stem Cell Pluripotency Laboratory, Max Planck Institute for Molecular Biomedicine, Münster, Germany; Department of Cardiovascular Medicine, Institute of Genetics of Heart Diseases, University of Münster Medical School, Münster, Germany.

出版信息

Stem Cell Res. 2017 May;21:26-28. doi: 10.1016/j.scr.2017.03.015. Epub 2017 Mar 23.

Abstract

Loss-of-function mutations in the PITX2 transcription factor gene have been shown to cause familial atrial fibrillation (AF). To potentially model aspects of AF and unravel PITX2-regulated downstream genes for drug target discovery, we here report the generation of integration-free PITX2-deficient hiPS cell lines. We also show that both PITX2 knockout hiPS cells and isogenic wild-type controls can selectively be differentiated into human atrial cardiomyocytes, to potentially uncover differentially expressed gene sets between these groups.

摘要

PITX2转录因子基因的功能丧失突变已被证明会导致家族性心房颤动(AF)。为了潜在地模拟AF的各个方面并揭示PITX2调控的下游基因以进行药物靶点发现,我们在此报告了无整合的PITX2缺陷型人诱导多能干细胞系的产生。我们还表明,PITX2基因敲除的人诱导多能干细胞和同基因野生型对照都可以选择性地分化为人心房心肌细胞,以潜在地揭示这些组之间差异表达的基因集。

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