Targeting epigenetics in sarcomas through EZH2 inhibition

Soft-tissue sarcomas represent a heterogeneous group of diseases with distinct genetic and clinical features accounting for up to 1% of cancer in adults and 15% of cancer in children. Epithelioid sarcoma is an extremely rare and aggressive tumor affecting young adults that is characterized by loss o...

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Autor principal: Italiano, Antoine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Editorial
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7140314/
https://www.ncbi.nlm.nih.gov/pubmed/32264965
http://dx.doi.org/10.1186/s13045-020-00868-4
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author Italiano, Antoine
author_facet Italiano, Antoine
author_sort Italiano, Antoine
collection PubMed
description Soft-tissue sarcomas represent a heterogeneous group of diseases with distinct genetic and clinical features accounting for up to 1% of cancer in adults and 15% of cancer in children. Epithelioid sarcoma is an extremely rare and aggressive tumor affecting young adults that is characterized by loss of INI1 expression. INI1 (SMARCB1, SNF5, BAF47) is a subunit of the SWI/SNF chromatin remodeling complex that opposes the enzymatic function of EZH2. When INI1 loses its regulatory function, EZH2 activity is de-regulated, allowing EZH2 to play a driving, oncogenic role. Tazemetostat, a specific EZH2 inhibitor, has just been approved for patients with advanced epithelioid sarcoma and represents a new therapeutic option in this devastating disease.
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spelling pubmed-71403142020-04-11 Targeting epigenetics in sarcomas through EZH2 inhibition Italiano, Antoine J Hematol Oncol Editorial Soft-tissue sarcomas represent a heterogeneous group of diseases with distinct genetic and clinical features accounting for up to 1% of cancer in adults and 15% of cancer in children. Epithelioid sarcoma is an extremely rare and aggressive tumor affecting young adults that is characterized by loss of INI1 expression. INI1 (SMARCB1, SNF5, BAF47) is a subunit of the SWI/SNF chromatin remodeling complex that opposes the enzymatic function of EZH2. When INI1 loses its regulatory function, EZH2 activity is de-regulated, allowing EZH2 to play a driving, oncogenic role. Tazemetostat, a specific EZH2 inhibitor, has just been approved for patients with advanced epithelioid sarcoma and represents a new therapeutic option in this devastating disease. BioMed Central 2020-04-07 /pmc/articles/PMC7140314/ /pubmed/32264965 http://dx.doi.org/10.1186/s13045-020-00868-4 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Editorial
Italiano, Antoine
Targeting epigenetics in sarcomas through EZH2 inhibition
title Targeting epigenetics in sarcomas through EZH2 inhibition
title_full Targeting epigenetics in sarcomas through EZH2 inhibition
title_fullStr Targeting epigenetics in sarcomas through EZH2 inhibition
title_full_unstemmed Targeting epigenetics in sarcomas through EZH2 inhibition
title_short Targeting epigenetics in sarcomas through EZH2 inhibition
title_sort targeting epigenetics in sarcomas through ezh2 inhibition
topic Editorial
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7140314/
https://www.ncbi.nlm.nih.gov/pubmed/32264965
http://dx.doi.org/10.1186/s13045-020-00868-4
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