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Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications

Roberta Ciarapica1*, Lucio Miele2, Antonio Giordano34, Franco Locatelli15 and Rossella Rota1*

Author affiliations

1 Department of Oncohematology, IRCCS, Ospedale Pediatrico Bambino Gesù, Roma, Italy

2 Cancer Institute, University of Mississippi Medical Center, Jackson, MI, USA

3 Sbarro Institute for Cancer Research and Molecular Medicine, Temple University, Philadelphia, PA, USA

4 Department of Human Pathology and Oncology, Università of Siena, Siena, Italy

5 Dipartimento di Scienze Pediatriche, Università di Pavia, Pavia, Italy

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Citation and License

BMC Medicine 2011, 9:63  doi:10.1186/1741-7015-9-63

Published: 25 May 2011

Abstract

Soft tissue sarcomas of childhood are a group of heterogeneous tumors thought to be derived from mesenchymal stem cells. Surgical resection is effective only in about 50% of cases and resistance to conventional chemotherapy is often responsible for treatment failure. Therefore, investigations on novel therapeutic targets are of fundamental importance. Deregulation of epigenetic mechanisms underlying chromatin modifications during stem cell differentiation has been suggested to contribute to soft tissue sarcoma pathogenesis. One of the main elements in this scenario is enhancer of zeste homolog 2 (EZH2), a methyltransferase belonging to the Polycomb group proteins. EZH2 catalyzes histone H3 methylation on gene promoters, thus repressing genes that induce stem cell differentiation to maintain an embryonic stem cell signature. EZH2 deregulated expression/function in soft tissue sarcomas has been recently reported. In this review, an overview of the recently reported functions of EZH2 in soft tissue sarcomas is given and the hypothesis that its expression might be involved in soft tissue sarcomagenesis is discussed. Finally, the therapeutic potential of epigenetic therapies modulating EZH2-mediated gene repression is considered.

Keywords:
EZH2; soft tissue sarcomas; epigenetics; methylation; methyltransferases