Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant

The iPSC line NIMHi013-A was generated from peripheral blood mononuclear cells of a paediatric patient with drug resistant epilepsy. The proband was found to have a likely pathogenic missense variant in the SCN1A gene in heterozygous state, which segregated in the affected in dominant fashion. The v...

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Main Authors: Gautham Arunachal, Madhura Milind Nimonkar, Pavithra Mahadeva, Ramya Sukrutha, Kenchaiah Raghavendra, Ghati K. Chetan, Manjunatha M. Venkataswamy, Bhupesh Mehta, Yogananda S. Markandeya
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S187350612400271X
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author Gautham Arunachal
Madhura Milind Nimonkar
Pavithra Mahadeva
Ramya Sukrutha
Kenchaiah Raghavendra
Ghati K. Chetan
Manjunatha M. Venkataswamy
Bhupesh Mehta
Yogananda S. Markandeya
author_facet Gautham Arunachal
Madhura Milind Nimonkar
Pavithra Mahadeva
Ramya Sukrutha
Kenchaiah Raghavendra
Ghati K. Chetan
Manjunatha M. Venkataswamy
Bhupesh Mehta
Yogananda S. Markandeya
author_sort Gautham Arunachal
collection DOAJ
description The iPSC line NIMHi013-A was generated from peripheral blood mononuclear cells of a paediatric patient with drug resistant epilepsy. The proband was found to have a likely pathogenic missense variant in the SCN1A gene in heterozygous state, which segregated in the affected in dominant fashion. The variant is in the Nav1.1 subunit of the voltage gated sodium ion channel. The iPSCs were generated using Sendai virus-based reprogramming. These iPSCs express pluripotent markers, present a normal karyotype and could differentiate into three germ layers. The iPSC line NIMHi013-A can be used to investigate epileptogenesis in vitro.
format Article
id doaj-art-deabda466fcf4e829b780cfbaf70fb00
institution DOAJ
issn 1873-5061
language English
publishDate 2024-12-01
publisher Elsevier
record_format Article
series Stem Cell Research
spelling doaj-art-deabda466fcf4e829b780cfbaf70fb002025-08-20T02:49:53ZengElsevierStem Cell Research1873-50612024-12-018110357310.1016/j.scr.2024.103573Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variantGautham Arunachal0Madhura Milind Nimonkar1Pavithra Mahadeva2Ramya Sukrutha3Kenchaiah Raghavendra4Ghati K. Chetan5Manjunatha M. Venkataswamy6Bhupesh Mehta7Yogananda S. Markandeya8Department of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, Karnataka, IndiaDepartment of Biophysics, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, IndiaDepartment of Neurovirology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, IndiaDepartment of Human Genetics, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, Karnataka, IndiaDepartment of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, IndiaDepartment of Biophysics, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, IndiaDepartment of Neurovirology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, IndiaDepartment of Biophysics, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, IndiaDepartment of Biophysics, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, India; Corresponding author.The iPSC line NIMHi013-A was generated from peripheral blood mononuclear cells of a paediatric patient with drug resistant epilepsy. The proband was found to have a likely pathogenic missense variant in the SCN1A gene in heterozygous state, which segregated in the affected in dominant fashion. The variant is in the Nav1.1 subunit of the voltage gated sodium ion channel. The iPSCs were generated using Sendai virus-based reprogramming. These iPSCs express pluripotent markers, present a normal karyotype and could differentiate into three germ layers. The iPSC line NIMHi013-A can be used to investigate epileptogenesis in vitro.http://www.sciencedirect.com/science/article/pii/S187350612400271X
spellingShingle Gautham Arunachal
Madhura Milind Nimonkar
Pavithra Mahadeva
Ramya Sukrutha
Kenchaiah Raghavendra
Ghati K. Chetan
Manjunatha M. Venkataswamy
Bhupesh Mehta
Yogananda S. Markandeya
Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant
Stem Cell Research
title Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant
title_full Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant
title_fullStr Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant
title_full_unstemmed Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant
title_short Generation of induced pluripotent stem cell line, NIMHi013-A, from PBMCs of a female child with epilepsy carrying a novel SCN1A variant
title_sort generation of induced pluripotent stem cell line nimhi013 a from pbmcs of a female child with epilepsy carrying a novel scn1a variant
url http://www.sciencedirect.com/science/article/pii/S187350612400271X
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