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http://hdl.handle.net/2445/184276
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DC Field | Value | Language |
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dc.contributor.author | Mazuelas, Helena | - |
dc.contributor.author | Magallón Lorenz, Míriam | - |
dc.contributor.author | Fernández Rodríguez, Juana | - |
dc.contributor.author | Uriarte Arrazola, Itziar | - |
dc.contributor.author | Richaud Patin, Yvonne | - |
dc.contributor.author | Terribas, Ernest | - |
dc.contributor.author | Villanueva Garatachea, Alberto | - |
dc.contributor.author | Castellanos, Elisabeth | - |
dc.contributor.author | Blanco Guillermo, Ignacio | - |
dc.contributor.author | Raya Chamorro, Ángel | - |
dc.contributor.author | Chojnacki, Jakub | - |
dc.contributor.author | Heyn, Holger | - |
dc.contributor.author | Romagosa Pérez-Portabella, Cleofé | - |
dc.contributor.author | Lázaro García, Conxi | - |
dc.contributor.author | Gel, Bernat | - |
dc.contributor.author | Carrió, Meritxell | - |
dc.contributor.author | Serra Arenas, Eduard | - |
dc.date.accessioned | 2022-03-22T10:22:23Z | - |
dc.date.available | 2022-03-22T10:22:23Z | - |
dc.date.issued | 2022-02-01 | - |
dc.identifier.issn | 2211-1247 | - |
dc.identifier.uri | http://hdl.handle.net/2445/184276 | - |
dc.description.abstract | Plexiform neurofibromas (pNFs) are developmental tumors that appear in neurofibromatosis type 1 individuals, constituting a major source of morbidity and potentially transforming into a highly metastatic sarcoma (MPNST). pNFs arise after NF1 inactivation in a cell of the neural crest (NC)-Schwann cell (SC) lineage. Here, we develop an iPSC-based NC-SC in vitro differentiation system and construct a lineage expression road map for the analysis of different 2D and 3D NF models. The best model consists of generating heterotypic spheroids (neurofibromaspheres) composed of iPSC-derived differentiating NF1((-/-)) SCs and NF1((+/-)) pNF-derived fibroblasts (Fbs). Neurofibromaspheres form by maintaining highly proliferative NF1((-/-)) cells committed to the NC-SC axis due to SC-SC and SC-Fb interactions, resulting in SC linage cells at different maturation points. Upon engraftment on the mouse sciatic nerve, neurofibromaspheres consistently generate human NF-like tumors. Analysis of expression roadmap genes in human pNF single-cell RNAseq data uncovers the presence of SC subpopulations at distinct differentiation states. | - |
dc.format.extent | 25 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Elsevier BV | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1016/j.celrep.2022.110385 | - |
dc.relation.ispartof | Cell Reports, 2022, vol 38, num 7 | - |
dc.relation.uri | https://doi.org/10.1016/j.celrep.2022.110385 | - |
dc.rights | cc by-nc-nd (c) Mazuelas, Helena et al, 2022 | - |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/es/ | * |
dc.source | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) | - |
dc.subject.classification | Neurofibromatosi | - |
dc.subject.classification | Genètica | - |
dc.subject.other | Neurofibromatosis | - |
dc.subject.other | Genetics | - |
dc.title | Modeling iPSC-derived human neurofibroma-like tumors in mice uncovers the heterogeneity of Schwann cells within plexiform neurofibromas | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.date.updated | 2022-03-22T08:33:45Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 35172160 | - |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
Files in This Item:
File | Description | Size | Format | |
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PIIS2211124722001061.pdf | 7.83 MB | Adobe PDF | View/Open |
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