A human embryonic stem cell-based model reveals the cell of origin of FOXR2-activated CNS neuroblastoma.

ABSTRACT:

Background

FOXR2-activated central nervous system (CNS) neuroblastoma (CNS NB-FOXR2) is a recently identified subtype of brain tumor characterized by the elevated expression of the transcription factor FOXR2 mainly due to genomic rearrangements. However, the precise pathogenic mechanisms, including the cell type of origin, remain elusive.

Methods

A gene expression analysis of patient tumors was performed to identify putative cell types of origin. Based on this prediction, a new human embryonic stem cell-based model was developed to validate the origin and to examine the molecular and cellular mechanisms underlying the formation of CNS NB-FOXR2.

Results

Our data showed that CNS NB-FOXR2 tumors express a high level of lineage marker genes associated with the medial gang

SUBMITTER: Royston HN 

PROVIDER: S-EPMC11364937 | biostudies-literature | 2024 Jan-Dec

REPOSITORIES: biostudies-literature