SOX2 commands LIM homeobox transcription factors in choroid plexus development and tumorigenesis.

ABSTRACT:

Background

Choroid plexus (CP) tumors are rare brain neoplasms that mainly affect the pediatric population. Unlike benign CP papilloma (CPP), CP carcinoma (CPC) is an aggressive cancer with a dismal survival rate. Despite chromosome-wide rearrangements, drivers of most CP tumors remain elusive except recurrent alterations in TP53. Studies of signaling dysregulation may bring biological understanding of these malignancies. Previous studies implicated NOTCH signaling in CP tumors; we developed mouse models of CP tumors driven by NOTCH activation and Trp53 loss, respectively. This work examined the role of the transcription factor SOX2 in CP development and tumorigenesis.

Methods

Multi-omics approaches were used to characterize cellular heterogeneity in NOTCH-driven CP tumors.

SUBMITTER: Faltings LJ 

PROVIDER: S-EPMC12448815 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature