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Generation of an induced pluripotent stem cell line from a patient with arrhythmogenic right ventricular cardiomyopathy harboring a TMEM43 splice-site variant

Authors :
Sun-Ho Lee
Gibbeum Lim
Hyoeun Kim
David Suh
Hyo-Kyoung Choi
Hyoung-Pyo Kim
Ho-Geun Yoon
Sahng Wook Park
Seok-Min Kang
Chulan Kwon
Jaewon Oh
Seung-Hyun Lee
Source :
Stem Cell Research, Vol 78, Iss , Pp 103453- (2024)
Publication Year :
2024
Publisher :
Elsevier, 2024.

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a cardiomyopathy that is predominantly inherited and characterized by cardiac arrhythmias and structural abnormalities. TMEM43 (transmembrane protein 43) is one of the well-known genetic culprits behind ACM. In this study, we successfully generated an induced pluripotent stem cell (iPSC) line, YCMi010-A, derived from a male patient diagnosed with ACM. Although these iPSCs harbored a heterozygous intronic splice variant, TMEM43 c.443-2A > G, they still displayed normal cellular morphology and were confirmed to express pluripotency markers. YCMi010-A iPSC line is a promising model for investigating the pathomechanisms associated with ACM and exploring potential therapeutic strategies.

Details

Language :
English
ISSN :
18735061
Volume :
78
Issue :
103453-
Database :
Directory of Open Access Journals
Journal :
Stem Cell Research
Publication Type :
Academic Journal
Accession number :
edsdoj.34be0af1e03449ec9c8e04a83a69f3dc
Document Type :
article
Full Text :
https://doi.org/10.1016/j.scr.2024.103453