Sun Liqiang, Li Jing, Li En, Niu Shaohui, Qin Zhiping, Zhi Qing, Zhao Jiajia, Xiong Haiyan, Li Yuan, Jian Liguo, Zhang Lihua
Department of Cardiology, The Second Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Department of Nuclear Medicine, The Second Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Stem Cell Res. 2020 Dec;49:102077. doi: 10.1016/j.scr.2020.102077. Epub 2020 Nov 4.
The role of muscle LIM protein (MLP), encoded by CSRP3, is not well understood in humans. CSRP3 knockout mice developed dilated cardiomyopathy with hypertrophy and heart failure after birth. Using CRISPR/Cas9, we generated an MLP deficient human ESC line WAe009-A-41 carrying a compound heterozygous 13 bp deletion/1 bp insertion in the CSRP3 gene. The WAe009-A-41 line exhibited a normal female karyotype (46, XX), expressed pluripotency markers and exhibited capability to differentiate into the three germ layers in vitro. MLP expression was not detectable in WAe009-A-41 line. This cell line can be a useful tool for studying the role of CSRP3 in cardiomyopathy and heart failure.
由CSRP3编码的肌肉LIM蛋白（MLP）在人类中的作用尚未完全明确。CSRP3基因敲除小鼠在出生后会发展为伴有肥大和心力衰竭的扩张型心肌病。利用CRISPR/Cas9技术，我们构建了一个MLP缺陷的人类胚胎干细胞系WAe009-A-41，该细胞系在CSRP3基因中携带复合杂合13bp缺失/1bp插入。WAe009-A-41细胞系呈现正常女性核型（46, XX），表达多能性标志物，并在体外表现出分化为三个胚层的能力。在WAe009-A-41细胞系中未检测到MLP表达。该细胞系可作为研究CSRP3在心肌病和心力衰竭中作用的有用工具。
