Targeting epigenetics in sarcomas through EZH2 inhibition
Abstract 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...
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| Format: | Article |
| Language: | English |
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BMC
2020-04-01
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| Series: | Journal of Hematology & Oncology |
| Online Access: | http://link.springer.com/article/10.1186/s13045-020-00868-4 |
| _version_ | 1818256828109160448 |
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| author | Antoine Italiano |
| author_facet | Antoine Italiano |
| author_sort | Antoine Italiano |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-12T17:33:57Z |
| format | Article |
| id | doaj.art-678c2286150140b281331ea6d4b847b8 |
| institution | Directory Open Access Journal |
| issn | 1756-8722 |
| language | English |
| last_indexed | 2024-12-12T17:33:57Z |
| publishDate | 2020-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Hematology & Oncology |
| spelling | doaj.art-678c2286150140b281331ea6d4b847b82022-12-22T00:17:16ZengBMCJournal of Hematology & Oncology1756-87222020-04-011311310.1186/s13045-020-00868-4Targeting epigenetics in sarcomas through EZH2 inhibitionAntoine Italiano0Institut Bergonié, Early Phase Trial and Sarcoma UnitsAbstract 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.http://link.springer.com/article/10.1186/s13045-020-00868-4 |
| spellingShingle | Antoine Italiano Targeting epigenetics in sarcomas through EZH2 inhibition Journal of Hematology & Oncology |
| 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 |
| url | http://link.springer.com/article/10.1186/s13045-020-00868-4 |
| work_keys_str_mv | AT antoineitaliano targetingepigeneticsinsarcomasthroughezh2inhibition |