Establishment of a GFP::LMNB1 knockin cell line (CSUi002-A-1) from a dystonia patient-specific iPSC by CRISPR/Cas9 editing.
Establishment of a GFP::LMNB1 knockin cell line (CSUi002-A-1) from a dystonia patient-specific iPSC by CRISPR/Cas9 editing.
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DOI：
10.1016/j.scr.2021.102505
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发表时间：
2021-08
影响因子：
1.2
通讯作者：
Yu Tang;Jie Ren;Chuan-Chang Li
中科院分区：
文献类型：
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作者：
Yu Tang;Jie Ren;Chuan-Chang Li
LMNB1作为核膜的主要成分之一，锚定异染色质，参与转录调控。LMNB1在DYT1肌张力障碍特异性神经元中表达上调，核浆错位。在这里，我们通过CRISPR/Cas9编辑，从DYT1患者来源的IPSC系中建立了一株融合表达GFP：：LMNB1的敲门细胞系。产生的IPSCs显示GFP和LMNB1共定位，这让人想起成功的基因组编辑。它们保持了多能和正常的核型，并具有分化为三个胚层的潜力。这一GFP：：LMNB1敲打蛋白IPSC将被用于研究DYT1肌张力障碍的椎板病理生理学以及其他以核为中心的问题。
LMNB1, as one of the major components of nuclear lamina, anchors heterochromatin and associates with transcription regulation. LMNB1 was previously demonstrated to be upregulated and nuclear-to-cytoplasmic mislocalized in DYT1 dystonia specific neurons. Here, we established a knockin cell line with GFP::LMNB1 fusion expression from a DYT1 patient derived iPSC line, by CRISPR/Cas9 editing. The generated iPSCs displayed GFP and LMNB1 co-localization, reminiscent of successful genomic editing. They remained pluripotent and normal karyotype, and possessed the potential to differentiate into three germ layers. This GFP::LMNB1 knockin iPSC will be used for studying the lamina-pathophysiology of DYT1 dystonia, and other nucleus-centered questions.