Comparison of three congruent patient-specific cell types for the modelling of a human genetic Schwann-cell disorder
DC Field | Value | Language |
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dc.contributor.author | Mukherjee-Clavin, Bipasha | - |
dc.contributor.author | Mi, Ruifa | - |
dc.contributor.author | Kern, Barbara | - |
dc.contributor.author | Choi, In Young | - |
dc.contributor.author | Lim, Hotae | - |
dc.contributor.author | Oh, Yohan | - |
dc.contributor.author | Lannon, Benjamin | - |
dc.contributor.author | Kim, Kevin J. | - |
dc.contributor.author | Bell, Shaughn | - |
dc.contributor.author | Hur, Junho K. | - |
dc.contributor.author | Hwang, Woochang | - |
dc.contributor.author | Che, Young Hyun | - |
dc.contributor.author | Habib, Omer | - |
dc.contributor.author | Baloh, Robert H. | - |
dc.contributor.author | Eggan, Kevin | - |
dc.contributor.author | Brandacher, Gerald | - |
dc.contributor.author | Hoke, Ahmet | - |
dc.contributor.author | Studer, Lorenz | - |
dc.contributor.author | Kim, Yong Jun | - |
dc.contributor.author | Lee, Gabsang | - |
dc.date.accessioned | 2022-07-09T13:56:32Z | - |
dc.date.available | 2022-07-09T13:56:32Z | - |
dc.date.created | 2021-05-11 | - |
dc.date.issued | 2019-07 | - |
dc.identifier.issn | 2157-846X | - |
dc.identifier.uri | https://scholarworks.bwise.kr/hanyang/handle/2021.sw.hanyang/147517 | - |
dc.description.abstract | Patient-specific human-induced pluripotent stem cells (hiPSCs) hold great promise for the modelling of genetic disorders. However, these cells display wide intra-and interindividual variations in gene expression, which makes distinguishing true-positive and false-positive phenotypes challenging. Data from hiPSC phenotypes and human embryonic stem cells (hESCs) harbouring the same disease mutation are also lacking. Here, we report a comparison of the molecular, cellular and functional characteristics of three congruent patient-specific cell types-hiPSCs, hESCs and direct-lineage-converted cells-derived from currently available differentiation and direct-reprogramming technologies for use in the modelling of Charcot-Marie-Tooth 1A, a human genetic Schwann-cell disorder featuring a 1.4 Mb chromosomal duplication. We find that the chemokines C-X-C motif ligand chemokine-1 (CXCL1) and macrophage chemoattractant protein-1 (MCP1) are commonly upregulated in all three congruent models and in clinical patient samples. The development of congruent models of a single genetic disease using somatic cells from a common patient will facilitate the search for convergent phenotypes. | - |
dc.language | 영어 | - |
dc.language.iso | en | - |
dc.publisher | NATURE PUBLISHING GROUP | - |
dc.title | Comparison of three congruent patient-specific cell types for the modelling of a human genetic Schwann-cell disorder | - |
dc.type | Article | - |
dc.contributor.affiliatedAuthor | Oh, Yohan | - |
dc.contributor.affiliatedAuthor | Hur, Junho K. | - |
dc.identifier.doi | 10.1038/s41551-019-0381-8 | - |
dc.identifier.scopusid | 2-s2.0-85063996460 | - |
dc.identifier.wosid | 000474416500013 | - |
dc.identifier.bibliographicCitation | NATURE BIOMEDICAL ENGINEERING, v.3, no.7, pp.571 - 582 | - |
dc.relation.isPartOf | NATURE BIOMEDICAL ENGINEERING | - |
dc.citation.title | NATURE BIOMEDICAL ENGINEERING | - |
dc.citation.volume | 3 | - |
dc.citation.number | 7 | - |
dc.citation.startPage | 571 | - |
dc.citation.endPage | 582 | - |
dc.type.rims | ART | - |
dc.type.docType | Article | - |
dc.description.journalClass | 1 | - |
dc.description.isOpenAccess | N | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.relation.journalResearchArea | Engineering | - |
dc.relation.journalWebOfScienceCategory | Engineering, Biomedical | - |
dc.subject.keywordPlus | ASCORBIC-ACID TREATMENT | - |
dc.subject.keywordPlus | NF-KAPPA-B | - |
dc.subject.keywordPlus | MOUSE MODEL | - |
dc.subject.keywordPlus | STEM-CELLS | - |
dc.subject.keywordPlus | MYELIN | - |
dc.subject.keywordPlus | DIFFERENTIATION | - |
dc.subject.keywordPlus | EXPRESSION | - |
dc.subject.keywordPlus | RECEPTOR | - |
dc.subject.keywordPlus | RECRUITMENT | - |
dc.subject.keywordPlus | PHENOTYPE | - |
dc.identifier.url | https://www.nature.com/articles/s41551-019-0381-8 | - |
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