Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa

Glycogen storage disease type IXa (GSD IXa) is a rare genetic disorder characterized by phosphorylase kinase (PhK) deficiency, which leads to excessive glycogen accumulation in the liver. Urinary cells (UCs) were isolated from a GSD IXa patient and reprogrammed into induced pluripotent stem cells (i...

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Autores principales: Yongbo Shin, Seon Ju Mun, Jaeseo Lee, Kyung-Sook Chung, Misuk Kim, Chong Kun Cheon, Myung Jin Son
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Lenguaje:EN
Publicado: Elsevier 2021
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Acceso en línea:https://doaj.org/article/65004ed9fae84708a42b793961f434a6
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spelling oai:doaj.org-article:65004ed9fae84708a42b793961f434a62021-11-04T04:27:41ZGeneration of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa1873-506110.1016/j.scr.2021.102584https://doaj.org/article/65004ed9fae84708a42b793961f434a62021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S1873506121004311https://doaj.org/toc/1873-5061Glycogen storage disease type IXa (GSD IXa) is a rare genetic disorder characterized by phosphorylase kinase (PhK) deficiency, which leads to excessive glycogen accumulation in the liver. Urinary cells (UCs) were isolated from a GSD IXa patient and reprogrammed into induced pluripotent stem cells (iPSCs) using Sendai virus. The established iPSC line, KRIBBi003-A, exhibited pluripotency marker expression and a normal karyotype. The differentiation capacity of the cell line was confirmed by the differentiation of the three germ layers in vitro. The established iPSC line is a potential useful resource for disease modeling of GSD IXa.Yongbo ShinSeon Ju MunJaeseo LeeKyung-Sook ChungMisuk KimChong Kun CheonMyung Jin SonElsevierarticleLiverOrganoidsGSD IXaiPSCsUrinary cellsBiology (General)QH301-705.5ENStem Cell Research, Vol 57, Iss , Pp 102584- (2021)
institution DOAJ
collection DOAJ
language EN
topic Liver
Organoids
GSD IXa
iPSCs
Urinary cells
Biology (General)
QH301-705.5
spellingShingle Liver
Organoids
GSD IXa
iPSCs
Urinary cells
Biology (General)
QH301-705.5
Yongbo Shin
Seon Ju Mun
Jaeseo Lee
Kyung-Sook Chung
Misuk Kim
Chong Kun Cheon
Myung Jin Son
Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa
description Glycogen storage disease type IXa (GSD IXa) is a rare genetic disorder characterized by phosphorylase kinase (PhK) deficiency, which leads to excessive glycogen accumulation in the liver. Urinary cells (UCs) were isolated from a GSD IXa patient and reprogrammed into induced pluripotent stem cells (iPSCs) using Sendai virus. The established iPSC line, KRIBBi003-A, exhibited pluripotency marker expression and a normal karyotype. The differentiation capacity of the cell line was confirmed by the differentiation of the three germ layers in vitro. The established iPSC line is a potential useful resource for disease modeling of GSD IXa.
format article
author Yongbo Shin
Seon Ju Mun
Jaeseo Lee
Kyung-Sook Chung
Misuk Kim
Chong Kun Cheon
Myung Jin Son
author_facet Yongbo Shin
Seon Ju Mun
Jaeseo Lee
Kyung-Sook Chung
Misuk Kim
Chong Kun Cheon
Myung Jin Son
author_sort Yongbo Shin
title Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa
title_short Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa
title_full Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa
title_fullStr Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa
title_full_unstemmed Generation of human induced pluripotent stem cell line, KRIBBi003-A, from urinary cells of a patient with glycogen storage disease type IXa
title_sort generation of human induced pluripotent stem cell line, kribbi003-a, from urinary cells of a patient with glycogen storage disease type ixa
publisher Elsevier
publishDate 2021
url https://doaj.org/article/65004ed9fae84708a42b793961f434a6
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