Biofabrication of a three dimensional human-based personalized neurofibroma model
| Autor: | Vincent Roy, Lydia Touzel-Deschênes, Rémy Lamontagne, Stephan Saikali, Hélène T. Khuong, François Gros-Louis, Matthieu Talagas, Nicolas Dupré |
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| Rok vydání: | 2021 |
| Předmět: |
0106 biological sciences
congenital hereditary and neonatal diseases and abnormalities Neurofibromatosis 1 Morphogenesis Gene mutation Biology 01 natural sciences Applied Microbiology and Biotechnology Tissue engineering 010608 biotechnology medicine Tumor Microenvironment Neurofibroma Humans Neurofibromatosis Neurofibromin 1 010401 analytical chemistry General Medicine medicine.disease nervous system diseases 0104 chemical sciences Cell biology Crosstalk (biology) Mutation biology.protein Molecular Medicine Schwann Cells Biofabrication |
| Zdroj: | Biotechnology journalREFERENCES. 16(6) |
| ISSN: | 1860-7314 |
| Popis: | Neurofibromas are the most characteristic feature of neurofibromatosis type 1 (NF1), a multisystemic disorder caused by aberrations in the neurofibromin gene (NF1). Despite significant progress over the last several years in understanding this disease, a suitable in vitro model to better mimic neurofibroma formation and growth has yet to be described. There is therefore a need to establish an in vitro, three dimensional model that allows the incorporation of multicellular lineages and the modulation of the cellular microenvironment-known to be important for cellular crosstalk and distribution of soluble factors-to study neurofibroma biology and morphogenesis. A self-assembly approach was used to generate tissue-engineered skins (TES) in which patient-derived spheroids made of NF1-associated Schwann cells and fibroblasts were seeded. We describe the first in vitro three dimensional neurofibroma model-directly derived from NF1 patients presenting with histopathological features-having an ECM protein expression profile quite similar to that of a native tumor. We observed efficient incorporation, proliferation, and migration of spheroids within NF1-TES over time. This biotechnological approach could provide a unique tool for precision medicine targeting NF1 and for assessing the tumorigenic properties of each NF1 gene mutation linked to tumor formation. |
| Databáze: | OpenAIRE |
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