What type of study is this?

This is a In Vitro Study study.

What question did this study set out to answer?

This research aims to investigate the persistence of radial glial cells after differentiating NF2-mutant neuroepithelial stem cells.

March 14, 2026Open Access

STEM-05. Persistence of radial glia cells after differentiation of NF2-mutant human neuroepithelial stem (NES) cells

Key Points

This research aims to investigate the persistence of radial glial cells after differentiating NF2-mutant neuroepithelial stem cells.
Derived NF2 knockout human neuroepithelial stem cells using CRISPR-Cas9 editing.
Examined cellular persistence in vitro and in vivo.
Identified markers like SOX2, BLBP, and GFAP in radial glia-like cells.
Observed abnormal persistence of radial glia-like cells post-differentiation.
Identified key molecular pathways that may contribute to ependymoma progression.

Abstract

Abstract Ependymoma of the spinal cord (SP-EPN) occurs in up to 50% of patients with NF2-related schwannomatosis (NF2) and are thought to arise from the radial glia cells. Most tumors in the NF2 population are diagnosed on screening MRI scans in childhood, but effective medical treatment for early-stage tumors do not currently exist. To date, the only effective treatment option for progressive or clinically symptomatic ependymoma in older children and young adults is high-risk surgery. There is a critical need to better model systems to define the molecular pathways that lead to tumorigenesis, and to identify new drug targets that halt the progression of these cells at an early stage. Previously, we derived human neuroepithelial stem (NES) cell lines from embryonic hindbrain and induced pluripotent (iPS) cells. These cells represent the human neural tube at 5-6 weeks gestation and can be differentiated towards spinal progenitors. They can be genetically modified to examine the molecular and cellular consequences of NF2 mutations on progenitors of the developing brainstem/spinal cord. We present preliminary data showing the generation of neuroepithelial stem (NES) cells with NF2 knockout following CRISPR-Cas9 editing of the NF2 gene. We observed persistence of pre-neoplastic radial glia-like cells after differentiation of NF2 knockout neuroepithelial stem (NF2-/-) cells in vitro and the persistence of RG like cells in vivo. These cells display markers of of RG, such as expression of SOX2, BLBPand GFAP. The NF2-mutant NES cells (represented cells of 5-6 week human neural tube) fail to differentiate normally and appear to persist as aberrant radial glial cells. This neuroepithelial cell-derived model of preneoplastic growth may provide unique insight into the cellular origins of ependymoma, and may be an important system for the study of molecular pathways underpinning ependymoma precursors in NF2-related schwannomatosis.

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Cite This Study

Tailor et al. (Fri,) studied this question.

synapsesocial.com/papers/69b4ba3618185d8a39802e6e https://doi.org/https://doi.org/10.1093/neuped/wuaf001.308

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