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Full-Text Articles in Medicine and Health Sciences
Prc2 Is Recurrently Inactivated Through Eed Or Suz12 Loss In Malignant Peripheral Nerve Sheath Tumors, W. Lee, S. Teckie, T. Wiesner, A. Viale, S. Singer, D. Zheng, M. F. Berger, Y. Chen, C. R. Antonescu, P. Chi, +11 Additional Authors
Prc2 Is Recurrently Inactivated Through Eed Or Suz12 Loss In Malignant Peripheral Nerve Sheath Tumors, W. Lee, S. Teckie, T. Wiesner, A. Viale, S. Singer, D. Zheng, M. F. Berger, Y. Chen, C. R. Antonescu, P. Chi, +11 Additional Authors
Journal Articles
Malignant peripheral nerve sheath tumors (MPNSTs) represent a group of highly aggressive soft-tissue sarcomas that may occur sporadically, in association with neurofibromatosis type I (NF1 associated) or after radiotherapy. Using comprehensive genomic approaches, we identified loss-of-function somatic alterations of the Polycomb repressive complex 2 (PRC2) components (EED or SUZ12) in 92% of sporadic, 70% of NF1-associated and 90% of radiotherapy-associated MPNSTs. MPNSTs with PRC2 loss showed complete loss of trimethylation at lysine 27 of histone H3 (H3K27me3) and aberrant transcriptional activation of multiple PRC2-repressed homeobox master regulators and their regulated developmental pathways. Introduction of the lost PRC2 component in a …