期刊
STEM CELL REPORTS
卷 12, 期 2, 页码 411-426出版社
CELL PRESS
DOI: 10.1016/j.stemcr.2019.01.001
关键词
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资金
- Johns Hopkins University School of Medicine
- Neurofibromatosis Therapeutic Acceleration Program (NTAP)
- Spanish Ministry of Science and Innovation, Carlos III Health Institute (ISCIII) Plan Estatal de I. D. i 2013-16 [PI14/00577, PI17/00524, RD16/0011/0024, PIE14/00061]
- FEDER program
- Government of Catalonia [2017-SGR-899]
- CERCA Program/Generalitat de Catalunya
- ACNefi
- Spanish Association Against Cancer (AECC)
- NTAP Cell Culture Initiative
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.
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