Enhancer reprogramming in PRC2-deficient malignant peripheral nerve sheath tumors induces a targetable de-differentiated state

Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that frequently harbor genetic alterations in polycomb repressor complex 2 (PRC2) components— SUZ12 and EED . Here, we show that PRC2 loss confers a dedifferentiated early neural-crest phenotype which is exclusive to PRC2-mut...

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Veröffentlicht in:	Acta neuropathologica 2021-09, Vol.142 (3), p.565-590
Hauptverfasser:	Kochat, Veena, Raman, Ayush T., Landers, Sharon M., Tang, Ming, Schulz, Jonathan, Terranova, Christopher, Landry, Jace P., Bhalla, Angela D., Beird, Hannah C., Wu, Chia-Chin, Jiang, Yingda, Mao, Xizeng, Lazcano, Rossana, Gite, Swati, Ingram, Davis R., Yi, Min, Zhang, Jianhua, Keung, Emily Z., Scally, Christopher P., Roland, Christina L., Hunt, Kelly K., Feig, Barry W., Futreal, P. Andrew, Hwu, Patrick, Wang, Wei-Lien, Lazar, Alexander J., Slopis, John M., Wilson-Robles, Heather, Wiener, Dominique J., McCutcheon, Ian E., Wustefeld-Janssens, Brandan, Rai, Kunal, Torres, Keila E.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Biomarkers, Tumor Cell Cycle Proteins - antagonists & inhibitors Cell Differentiation - genetics Cell Line, Tumor Chromatin Comparative analysis Dogs Enhancer Elements, Genetic - genetics Enhancers Epigenesis, Genetic - genetics Epigenetic inheritance Epigenetics Ethylenediaminetetraacetic acid Genetic engineering Genotype & phenotype Homeodomain Proteins - genetics Humans Medicine Medicine & Public Health Mice Mice, Transgenic Mutants Mutation Nerve Sheath Neoplasms - drug therapy Nerve Sheath Neoplasms - genetics Nerve Sheath Neoplasms - pathology Neural crest Neural Crest - pathology Neurosciences Original Paper Pathology Peripheral nerves Peripheral Nervous System Neoplasms - drug therapy Peripheral Nervous System Neoplasms - genetics Peripheral Nervous System Neoplasms - pathology Phenotypes Polycomb group proteins Polycomb Repressive Complex 2 - genetics Sarcoma Species Specificity Transcription Factors - antagonists & inhibitors Transcription Factors - genetics Tumors Xenograft Model Antitumor Assays Zebrafish
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