DC Field | Value | Language |
---|---|---|
dc.contributor.author | Ju, Younghee | ko |
dc.contributor.author | Park, Jun Sung | ko |
dc.contributor.author | Kim, Daejeong | ko |
dc.contributor.author | Kim, Bumsoo | ko |
dc.contributor.author | Lee, Jeong Ho | ko |
dc.contributor.author | Nam, Yoonkey | ko |
dc.contributor.author | Yoo, Han-Wook | ko |
dc.contributor.author | Lee, Beom Hee | ko |
dc.contributor.author | Han, Yong-Mahn | ko |
dc.date.accessioned | 2020-07-18T00:56:12Z | - |
dc.date.available | 2020-07-18T00:56:12Z | - |
dc.date.created | 2020-07-10 | - |
dc.date.created | 2020-07-10 | - |
dc.date.created | 2020-07-10 | - |
dc.date.created | 2020-07-10 | - |
dc.date.issued | 2020-07 | - |
dc.identifier.citation | STEM CELL RESEARCH THERAPY, v.11, no.1 | - |
dc.identifier.issn | 1757-6512 | - |
dc.identifier.uri | http://hdl.handle.net/10203/275496 | - |
dc.description.abstract | BackgroundNoonan syndrome (NS) is a developmental disorder caused by mutations of Src homology 2 domain-containing protein tyrosine phosphatase 2 (SHP2). Although NS patients have diverse neurological manifestations, the mechanisms underlying the involvement of SHP2 mutations in neurological dysfunction remain elusive.MethodsInduced pluripotent stem cells generated from dermal fibroblasts of three NS-patients (NS-iPSCs) differentiated to the neural cells by using two different culture systems, 2D- and 3D-cultured systems in vitro.ResultsHere we represent that SHP2 mutations cause aberrant neural development. The NS-iPSCs exhibited impaired development of EBs in which BMP and TGF-beta signalings were activated. Defective early neuroectodermal development of NS-iPSCs recovered by inhibition of both signalings and further differentiated into NPCs. Intriguingly, neural cells developed from NS-NPCs exhibited abundancy of the glial cells, neurites of neuronal cells, and low electrophysiological property. Those aberrant phenotypes were also detected in NS-cerebral organoids. SHP2 inhibition in the NS-NPCs and NS-cerebral organoids ameliorated those anomalies such as biased glial differentiation and low neural activity.ConclusionOur findings demonstrate that SHP2 mutations contribute to precocious gliogenesis in NS-iPSCs during neural development in vitro. | - |
dc.language | English | - |
dc.publisher | BMC | - |
dc.title | SHP2 mutations induce precocious gliogenesis of Noonan syndrome-derived iPSCs during neural development in vitro (vol 11, 209, 2020) | - |
dc.type | Article | - |
dc.identifier.wosid | 000540235400001 | - |
dc.identifier.scopusid | 2-s2.0-85086007918 | - |
dc.type.rims | ART | - |
dc.citation.volume | 11 | - |
dc.citation.issue | 1 | - |
dc.citation.publicationname | STEM CELL RESEARCH THERAPY | - |
dc.identifier.doi | 10.1186/s13287-020-01709-4 | - |
dc.contributor.localauthor | Lee, Jeong Ho | - |
dc.contributor.localauthor | Nam, Yoonkey | - |
dc.contributor.localauthor | Han, Yong-Mahn | - |
dc.contributor.nonIdAuthor | Yoo, Han-Wook | - |
dc.contributor.nonIdAuthor | Lee, Beom Hee | - |
dc.description.isOpenAccess | Y | - |
dc.type.journalArticle | Article | - |
dc.subject.keywordAuthor | Noonan syndrome | - |
dc.subject.keywordAuthor | Induced pluripotent stem cells | - |
dc.subject.keywordAuthor | SHP2 mutations | - |
dc.subject.keywordAuthor | Neural development | - |
dc.subject.keywordAuthor | Gliogenesis | - |
dc.subject.keywordAuthor | Cerebral organoids | - |
dc.subject.keywordPlus | PLURIPOTENT STEM-CELLS | - |
dc.subject.keywordPlus | OF-FUNCTION MUTATIONS | - |
dc.subject.keywordPlus | PTPN11 MUTATIONS | - |
dc.subject.keywordPlus | SELF-RENEWAL | - |
dc.subject.keywordPlus | DIFFERENTIATION | - |
dc.subject.keywordPlus | GERMLINE | - |
dc.subject.keywordPlus | NEURONS | - |
dc.subject.keywordPlus | PATHWAY | - |
dc.subject.keywordPlus | ASTROGLIOGENESIS | - |
dc.subject.keywordPlus | PROLIFERATION | - |
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