用于体外和体内用途的人诱导多能干细胞衍生的神经纤维瘤球的生成。

Generation of human iPSC-derived neurofibromaspheres for in vitro and in vivo uses.

作者信息

Mazuelas Helena, Uriarte-Arrazola Itziar, Fernández-Rodríguez Juana, Magallón-Lorenz Miriam, Villanueva Alberto, Lázaro Conxi, Gel Bernat, Serra Eduard, Carrió Meritxell

机构信息

Hereditary Cancer Group, Germans Trias i Pujol Research Institute (IGTP), Can Ruti Campus, Badalona, 08916 Barcelona, Spain.

Hereditary Cancer Program, Catalan Institute of Oncology (ICO-IDIBELL), L'Hospitalet de Llobregat, 08098 Barcelona, Spain; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain.

出版信息

STAR Protoc. 2023 Mar 27;4(2):102198. doi: 10.1016/j.xpro.2023.102198.

DOI:10.1016/j.xpro.2023.102198

PMID:36976677

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10060107/

Abstract

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)..

摘要

神经纤维瘤是与1型神经纤维瘤病相关的良性外周神经系统肿瘤，起源于NF1（-/-）雪旺细胞前体。我们描述了一种通过将诱导多能干细胞分化而来的NF1（-/-）雪旺细胞与神经纤维瘤原代成纤维细胞结合来生成神经纤维瘤球的方案。我们还描述了将神经纤维瘤球植入裸鼠坐骨神经后神经纤维瘤样肿瘤的发展情况。该模型构成了一个用于药物筛选和神经纤维瘤生物学研究的通用平台。有关此方案的使用和执行的完整详细信息，请参考马祖拉斯等人（2022年）的研究。