Transdifferentiation of endothelial progenitor cells into rhabdomyosarcoma defined by hedgehog signaling competence.

ABSTRACT: Rhabdomyosarcoma (RMS) is a pediatric soft tissue sarcoma resembling developing skeletal muscle, but tumors are found in sites devoid of skeletal muscle, suggesting a non-myogenic cell of origin. We previously described an endothelial progenitor cell-derived model of RMS by activating a conditional, constitutively active Smoothened mutant (SmoM2) with aP2-Cre. We sought to further identify the endothelial progenitor cell of origin in our aP2-Cre;R26^SmoM2 model using mice with more endothelial-specific Cre recombinase expression. Using lineage tracing of embryonic endothelial progenitor cells and mature, adult endothelial cells, we show that a selected subset of endothelial progenitor cells can transform into RMS. These endothelial progenitor cells have a specific window during e

SUBMITTER: Gadek KE 

PROVIDER: S-EPMC12592795 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature