[HTML][HTML] Generation of human iPSC-derived neurofibromaspheres for in vitro and in vivo uses

H Mazuelas, I Uriarte-Arrazola, J Fernández-Rodríguez… - STAR protocols, 2023 - Elsevier
H Mazuelas, I Uriarte-Arrazola, J Fernández-Rodríguez, M Magallón-Lorenz, A Villanueva…
STAR protocols, 2023Elsevier
Neurofibromas are benign peripheral nervous system tumors associated with
neurofibromatosis type 1, which originate from NF1 (−/−) Schwann cell precursors. We
describe a protocol to generate neurofibromaspheres by differentiating NF1 (−/−) Schwann
cells from induced pluripotent stem cells and combining them with neurofibroma primary
fibroblasts. We also describe the development of neurofibroma-like tumors when
neurofibromaspheres are engrafted in the sciatic nerve of nude mice. This model constitutes …
Summary
Neurofibromas are benign peripheral nervous system tumors associated with neurofibromatosis type 1, which originate from NF1(−/−) Schwann cell precursors. We describe a protocol to generate neurofibromaspheres by differentiating NF1(−/−) Schwann cells from induced pluripotent stem cells and combining them with neurofibroma primary fibroblasts. We also describe the development of neurofibroma-like tumors when neurofibromaspheres are engrafted in the sciatic nerve of nude mice. This model constitutes a versatile platform for drug screening and the study of neurofibroma biology.
For complete details on the use and execution of this protocol, please refer to Mazuelas et al. (2022).1
Elsevier