Human induced pluripotent stem cell line ZZUNEUi027-A generated from a long QT syndrome patient with a heterozygous KCNH2 (c. 128 A > G) mutant

Jiangtao Zhao; Lu Wang; Kui Zhu; Xiaowei Chen; Fen Qin; Xiaowei Li; Jianzeng Dong; Hailong Tao

doi:10.1016/j.scr.2022.102836

Human induced pluripotent stem cell line ZZUNEUi027-A generated from a long QT syndrome patient with a heterozygous KCNH2 (c. 128 A > G) mutant

Stem Cell Res. 2022 Aug:63:102836. doi: 10.1016/j.scr.2022.102836. Epub 2022 Jun 7.

Authors

Jiangtao Zhao¹, Lu Wang², Kui Zhu¹, Xiaowei Chen¹, Fen Qin¹, Xiaowei Li², Jianzeng Dong³, Hailong Tao⁴

Affiliations

¹ Department of Cardiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
² Department of Cardiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Henan Key Laboratory of Hereditary Cardiovascular Diseases, Zhengzhou 450052, China.
³ Department of Cardiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Henan Key Laboratory of Hereditary Cardiovascular Diseases, Zhengzhou 450052, China; Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, National Clinical Research Centre for Cardiovascular Diseases, No. 2 Beijing Anzhen Road, Chaoyang District, Beijing 100029, China.
⁴ Department of Cardiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China. Electronic address: hailongtao@zzu.edu.cn.

PMID: 35700634
DOI: 10.1016/j.scr.2022.102836

Abstract

Long QT syndrome is one of the most common hereditary arrhythmias. Mutations in KCNH2 can cause long QT syndrome type 2 (LQT2). In this study, we generated a human induced pluripotent stem cell line ZZUNEUi027-A from a LQT2 female patient with c. 128A → G in KCNH2 gene using non-integrative Sendai viral reprogramming technology. This cell line expresses pluripotency markers, exhibits a normal female karyotype (46, XX) and could differentiate into all three germ layers in vitro. ZZUNEUi027-A can serve as a cell disease model in the understanding of LQT2 pathogenesis.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Cell Line
ERG1 Potassium Channel / genetics
ERG1 Potassium Channel / metabolism
Female
Heterozygote
Humans
Induced Pluripotent Stem Cells* / metabolism
Long QT Syndrome* / metabolism
Mutation / genetics

Substances

ERG1 Potassium Channel
KCNH2 protein, human