YAP1::TFE3 mediates endothelial-to-mesenchymal plasticity in epithelioid hemangioendothelioma.

ABSTRACT: The rare vascular sarcoma epithelioid hemangioendothelioma (EHE) is defined by WWTR1 or YAP1 gene rearrangements that result in functional fusion proteins. Previous studies have demonstrated the ability of these gene fusions to function as constitutively active TEAD coactivators, while also retaining the ability to drive transcription of canonical CAMTA1 or TFE3 genes, respectively. To better understand the biology underlying EHE, we generated EHE in vitro models using endothelial cell lines and found that inducible expression of YAP1::TFE3 (YT) caused a significant change in cellular plasticity. Specifically, YT expression led to endothelial-to-mesenchymal transition (EndMT), a process in which endothelial cells lose their highly specialized identity and gain expression of genes typically

SUBMITTER: Murphy A 

PROVIDER: S-EPMC12936420 | biostudies-literature | 2026 Feb

REPOSITORIES: biostudies-literature