Modeling tumors of the peripheral nervous system associated with Neurofibromatosis type 1: Reprogramming plexiform neurofibroma cells

被引：9

作者：

Mazuelas, Helena ^{[1
]}

Carrio, Meritxell ^{[1
]}

Serra, Eduard ^{[1
]}

机构：

[1] Germans Trias & Pujol Res Inst IGTP, Hereditary Canc Grp, Can Ruti Biomed Campus, Barcelona, Spain

来源：

STEM CELL RESEARCH | 2020年 / 49卷

关键词：

Cancer predisposition syndrome; Neurofibromatosis; Plexiform neurofibroma; iPSC; Neural crest; Schwann cell; Spheroid; PLURIPOTENT STEM-CELLS; NEURAL CREST CELLS; SCHWANN-CELLS; EPIGENETIC MEMORY; GLIAL-CELLS; DIRECTED DIFFERENTIATION; ATYPICAL NEUROFIBROMAS; TUMORIGENIC PROPERTIES; SHEATH TUMORS; SELF-RENEWAL;

D O I：

10.1016/j.scr.2020.102068

中图分类号：

Q813 [细胞工程];

学科分类号：

摘要：

Plexiform neurofibromas (pNFs) are benign tumors of the peripheral nervous system (PNS) that can progress towards a deadly soft tissue sarcoma termed malignant peripheral nerve sheath tumor (MPNST). pNFs appear during development in the context of the genetic disease Neurofibmmatosis type 1 (NF1) due to the complete loss of the NF1 tumor suppressor gene in a cell of the neural crest (NC) - Schwann cell (SC) axis of differentiation. NF1(-/-) cells from pNFs can be reprogrammed into induced pluripotent stem cells (iPSCs) that exhibit an increased proliferation rate and maintain full iPSC properties. Efficient protocols for iPSC differentiation towards NC and SC exist and thus NC cells can be efficiently obtained from NF1(-/-) iPSCs and further differentiated towards SCs. In this review, we will focus on the iPSC modeling of pNFs, including the reprogramming of primary pNF-derived cells, the properties of pNF-derived iPSCs, the capacity to differentiate towards the NC-SC lineage, and how well iPSC-derived NF1(-/-) SC spheroids recapitulate pNF-derived primary SCs. The potential uses of NF1(-/-) iPSCs in pNF modeling and a future outlook are discussed.

引用

页数：10

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