Skeletal muscle formation represents a complex of highly organized and specialized systems that are still not fully understood. Epigenetic systems underline embryonic development, maintenance of stemness, and progression of differentiation. Polycomb group proteins play the role of gene silencing of stemness markers that regulate muscle differentiation. Enhancer of Zeste EZH2 is the catalytic subunit of the complex that is able to trimethylate lysine 27 of histone H3 and induce silencing of the involved genes. In embryonal Rhabdomyosarcoma and several other tumors, EZH2 is often deregulated and, in some cases, is associated with tumor malignancy. This review explores the molecular processes underlying the failure of muscle differentiation with a focus on the PRC2 complex. These considerations could open new studies aimed at the development of new cutting-edge therapeutic strategies in the onset of Rhabdomyosarcoma.

中文翻译：

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PRC2：一种表观遗传多蛋白复合物，在横纹肌肉瘤致癌过程中发挥关键作用

骨骼肌的形成代表了一个高度组织化和专业化的系统的复合体，但目前尚未完全了解。表观遗传系统强调胚胎发育、干性维持和分化进展。多梳蛋白发挥调节肌肉分化的干性标记基因沉默的作用。Zeste EZH2 的增强子是复合物的催化亚基，能够使组蛋白 H3 的赖氨酸 27 三甲基化并诱导相关基因沉默。在胚胎性横纹肌肉瘤和其他几种肿瘤中，EZH2 经常失调，并且在某些情况下与肿瘤恶性肿瘤相关。这篇综述探讨了肌肉分化失败的分子过程，重点关注 PRC2 复合体。这些考虑因素可能会开启新的研究，旨在开发针对横纹肌肉瘤发病的新的尖端治疗策略。