Establishment of a GFP::LMNB1 knockin cell line (CSUi002-A-1) from a dystonia patient-specific iPSC by CRISPR/Cas9 editing.
Stem Cell Res
; 55: 102505, 2021 08.
Article
em En
| MEDLINE
| ID: mdl-34438319
ABSTRACT
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 GFPLMNB1 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 GFPLMNB1 knockin iPSC will be used for studying the lamina-pathophysiology of DYT1 dystonia, and other nucleus-centered questions.
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Base de dados:
MEDLINE
Assunto principal:
Distonia
/
Células-Tronco Pluripotentes Induzidas
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article