Generation of human induced pluripotent stem cell lines from a fetus with congenital long QT syndrome and her healthy parents

Summary

Long QT syndrome (LQTS) is a channelopathy that predisposes affected individuals to ventricular arrhythmias and cardiac arrest. Here, a human induced pluripotent stem cell (hiPSC) line was generated from amniotic fluid cells (AFCs) of a 32-week fetus diagnosed with LQTS. Additionally, two iPSC lines were generated from peripheral blood mononuclear cells (PBMCs) of the fetus's healthy biological parents. Genome sequencing revealed that the fetus with LQTS carried a de novo KCNH2 variant, c.1898A > G (p.Asn633Ser). All three iPSC lines demonstrated normal morphology, karyotyping, and pluripotency. These iPSC lines provide a valuable in vitro model for LQTS caused by KCNH2 mutations. Copyright © 2025 The Author(s). Published by Elsevier B.V. All rights reserved.

Authors Putra M, Cuneo BF, Chi C, Zhang Z, Prudell L, Song K
Journal Stem cell research
Publication Date 2025 Dec;89:103848
PubMed 41072104
PubMed Central PMC13499978
DOI 10.1016/j.scr.2025.103848

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