The perfect PTEN – transcriptional regulation by PTEN dictates sarcoma identity

Abstract

Fusion-negative rhabdomyosarcoma (FN-RMS) is molecularly heterogeneous with few universal alterations except for Phosphatase and tensin homolog (PTEN) promoter hypermethylation. We demonstrate that losing Pten in FN-RMS engages an aberrant transcriptional program key in tumor maintenance and cell identity. These results highlight the importance between transcriptional state, cell of origin, and genetic perturbation in tumorigenesis.

Keywords

• rhabdomyosarcoma; pten; pax7; dbx1; leiomyosarcoma; mouse models of cancer; core regulatory circuits; sarcoma ;pediatric cancer; cancer and development