Recent studies have identified repeated mutations in the core components of the polycomb repressor complex 2 (PRC2), embryonic ectoderm development protein (EED), and zeste 12 homolog inhibitor (SUZ12) in MPNST. In most MPNSTs, the identification of EED and SUZ12 mutations in the PRC2 component may suggest the unconventional carcinogenic activity of the complete component EZH2, and provide new opportunities for therapeutic intervention.
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