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Overactivation of SHH signaling during embryonic choroid plexus development induces a cascade of dedifferentiation and carcinoma formation.

ABSTRACT: Choroid Plexus Carcinomas are highly malignant tumors arising predominantly in very young children and are associated with poor survival. Details on tumor initiation as well as oncogenic events are largely unknown. We generated hGFAP-cre::lsl-MYCN::lsl-Gli2(N)::Tp5fl/fl mice which developed tumors by the transition from multiciliated, differentiated cells towards monociliated highly proliferating cells. We profiled these tumors using spatial transcriptomics and revealed details of the dedifferntiation cascade that leads to tumor formation, the relevance of SHH signaling and identified druggable targets for future therapies.

ORGANISM(S): Mus musculus

PROVIDER: GSE289052 | GEO | 2026/08/26

REPOSITORIES: GEO

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| S-EPMC12510407 | biostudies-literature