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

<p>Abstract</p> <p>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 fail...

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Main Authors: Giordano Antonio, Miele Lucio, Ciarapica Roberta, Locatelli Franco, Rota Rossella
Format: Article
Language:English
Published: BMC 2011-05-01
Series:BMC Medicine
Subjects:
Online Access:http://www.biomedcentral.com/1741-7015/9/63
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author Giordano Antonio
Miele Lucio
Ciarapica Roberta
Locatelli Franco
Rota Rossella
author_facet Giordano Antonio
Miele Lucio
Ciarapica Roberta
Locatelli Franco
Rota Rossella
author_sort Giordano Antonio
collection DOAJ
description <p>Abstract</p> <p>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.</p>
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spelling doaj.art-e041a3c213e5429aaa1e1bedbc4da2d62022-12-21T22:40:11ZengBMCBMC Medicine1741-70152011-05-01916310.1186/1741-7015-9-63Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implicationsGiordano AntonioMiele LucioCiarapica RobertaLocatelli FrancoRota Rossella<p>Abstract</p> <p>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.</p>http://www.biomedcentral.com/1741-7015/9/63EZH2soft tissue sarcomasepigeneticsmethylationmethyltransferases
spellingShingle Giordano Antonio
Miele Lucio
Ciarapica Roberta
Locatelli Franco
Rota Rossella
Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications
BMC Medicine
EZH2
soft tissue sarcomas
epigenetics
methylation
methyltransferases
title Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications
title_full Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications
title_fullStr Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications
title_full_unstemmed Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications
title_short Enhancer of zeste homolog 2 (EZH2) in pediatric soft tissue sarcomas: first implications
title_sort enhancer of zeste homolog 2 ezh2 in pediatric soft tissue sarcomas first implications
topic EZH2
soft tissue sarcomas
epigenetics
methylation
methyltransferases
url http://www.biomedcentral.com/1741-7015/9/63
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AT locatellifranco enhancerofzestehomolog2ezh2inpediatricsofttissuesarcomasfirstimplications
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