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http://hdl.handle.net/2445/171527
Title: | Reprogramming Captures the Genetic and Tumorigenic Properties of Neurofibromatosis Type 1 Plexiform Neurofibromas |
Author: | Carrió, Meritxell Mazuelas, Helena Richaud-Patin, Yvonne Gel, Bernat Terribas, Ernest Rosas, Imma Jimenez Delgado, Senda Biayna, Josep Vendredy, Leen Blanco Guillermo, Ignacio Castellanos, Elisabeth Lázaro García, Conxi Raya Chamorro, Ángel Serra, Eduard |
Keywords: | Neurofibromatosi Antioncogens Neurofibromatosis Antioncogenes |
Issue Date: | 12-Feb-2019 |
Publisher: | Cell Press |
Abstract: | Neurofibromatosis type 1 (NF1) is a tumor predisposition genetic disease caused by mutations in the NF1 tumor suppressor gene. Plexiform neurofibromas (PNFs) are benign Schwann cell (SC) tumors of the peripheral nerve sheath that develop through NF1 inactivation and can progress toward a malignant soft tissue sarcoma. There is a lack of non-perishable model systems to investigate PNF development. We reprogrammed PNF-derived NF1(-/-) cells, descendants from the tumor originating cell. These NF1(-/-)-induced pluripotent stem cells (iPSCs) captured the genomic status of PNFs and were able to differentiate toward neural crest stem cells and further to SCs. iPSC-derived NF1(-/-) SCs exhibited a continuous high proliferation rate, poor myelination ability, and a tendency to form 3D spheres that expressed the same markers as their PNF-derived primary SC counterparts. They represent a valuable model to study and treat PNFs. PNF-derived iPSC lines were banked for making them available. |
Note: | Reproducció del document publicat a: https://doi.org/10.1016/j.stemcr.2019.01.001 |
It is part of: | Stem Cell Reports, 2019, vol. 12, num. 2, p. 411-426 |
URI: | http://hdl.handle.net/2445/171527 |
Related resource: | https://doi.org/10.1016/j.stemcr.2019.01.001 |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) Articles publicats en revistes (Institut de Recerca Biomèdica (IRB Barcelona)) |
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CarrioM.pdf | 6.66 MB | Adobe PDF | View/Open |
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