Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation

Long QT syndrome type 2 (LQT2) is a heart disorder resulting from a loss-of-function mutation in the KCNH2 gene that causes loss of Kv11.1 channel function, potentially resulting in syncope, arrhythmias, and sudden death. We derived induced pluripotent stem cell line from PBMC of LQT2 patient carryi...

Full description

Saved in:
Bibliographic Details
Main Authors: Dasom Mun, Ji-Young Kang, Malgeum Park, Gyeongseo Yoo, Hyoeun Kim, Nuri Yun, You Mi Hwang, Boyoung Joung
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S1873506124002903
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1846125583681978368
author Dasom Mun
Ji-Young Kang
Malgeum Park
Gyeongseo Yoo
Hyoeun Kim
Nuri Yun
You Mi Hwang
Boyoung Joung
author_facet Dasom Mun
Ji-Young Kang
Malgeum Park
Gyeongseo Yoo
Hyoeun Kim
Nuri Yun
You Mi Hwang
Boyoung Joung
author_sort Dasom Mun
collection DOAJ
description Long QT syndrome type 2 (LQT2) is a heart disorder resulting from a loss-of-function mutation in the KCNH2 gene that causes loss of Kv11.1 channel function, potentially resulting in syncope, arrhythmias, and sudden death. We derived induced pluripotent stem cell line from PBMC of LQT2 patient carrying a variant of pathogenic variant (c.157G > A; p.Gly53Ser). The generation of iPSC lines was achieved using the non-integrative Sendai virus-mediated iPSC reprogramming method. The iPSC cell line exhibit pluripotency, normal karyotype, stem cell morphology, and differentiation capability, resulting a reliable cell source to study the effects of KCNH2 mutation in disease-specific cell types.
format Article
id doaj-art-eff471611fdb4b298b86d7cf4a3f0d1a
institution Kabale University
issn 1873-5061
language English
publishDate 2024-12-01
publisher Elsevier
record_format Article
series Stem Cell Research
spelling doaj-art-eff471611fdb4b298b86d7cf4a3f0d1a2024-12-13T10:56:55ZengElsevierStem Cell Research1873-50612024-12-0181103592Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutationDasom Mun0Ji-Young Kang1Malgeum Park2Gyeongseo Yoo3Hyoeun Kim4Nuri Yun5You Mi Hwang6Boyoung Joung7Division of Cardiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of KoreaDivision of Cardiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of KoreaDivision of Cardiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of KoreaDivision of Cardiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of KoreaDepartment of Biochemistry and Molecular Biology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of KoreaGNTPharma Science and Technology Center for Health, 85 Songdogwahak-ro, Yeonsu-gu, Incheon 21983, Republic of KoreaDepartment of Cardiology, St. Vincent’s Hospital, The Catholic University of Korea, 93 Jungbu-daero, Paldal-gu, Suwon 16247, Republic of Korea; Catholic Research Institute for Intractable Cardiovascular Disease (CRID), College of Medicine, The Catholic University of Korea, 93 Jungbu-daero, Paldal-gu, Suwon 16247, Republic of KoreaDivision of Cardiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of Korea; Corresponding author.Long QT syndrome type 2 (LQT2) is a heart disorder resulting from a loss-of-function mutation in the KCNH2 gene that causes loss of Kv11.1 channel function, potentially resulting in syncope, arrhythmias, and sudden death. We derived induced pluripotent stem cell line from PBMC of LQT2 patient carrying a variant of pathogenic variant (c.157G > A; p.Gly53Ser). The generation of iPSC lines was achieved using the non-integrative Sendai virus-mediated iPSC reprogramming method. The iPSC cell line exhibit pluripotency, normal karyotype, stem cell morphology, and differentiation capability, resulting a reliable cell source to study the effects of KCNH2 mutation in disease-specific cell types.http://www.sciencedirect.com/science/article/pii/S1873506124002903
spellingShingle Dasom Mun
Ji-Young Kang
Malgeum Park
Gyeongseo Yoo
Hyoeun Kim
Nuri Yun
You Mi Hwang
Boyoung Joung
Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation
Stem Cell Research
title Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation
title_full Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation
title_fullStr Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation
title_full_unstemmed Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation
title_short Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation
title_sort establishment of a human induced pluripotent stem cell line from a long qt syndrome type 2 patient harboring a kcnh2 mutation
url http://www.sciencedirect.com/science/article/pii/S1873506124002903
work_keys_str_mv AT dasommun establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT jiyoungkang establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT malgeumpark establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT gyeongseoyoo establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT hyoeunkim establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT nuriyun establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT youmihwang establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation
AT boyoungjoung establishmentofahumaninducedpluripotentstemcelllinefromalongqtsyndrometype2patientharboringakcnh2mutation