Synovial sarcoma: when epigenetic changes dictate tumour development.

Details

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State: Public
Version: Final published version
Serval ID
serval:BIB_9F54855F01B8
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Synovial sarcoma: when epigenetic changes dictate tumour development.
Journal
Swiss medical weekly
Author(s)
Riggi N., Cironi L., Stamenkovic I.
ISSN
1424-3997 (Electronic)
ISSN-L
0036-7672
Publication state
Published
Issued date
19/11/2018
Peer-reviewed
Oui
Volume
148
Pages
w14667
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: epublish
Abstract
Synovial sarcoma is a highly aggressive soft tissue malignancy that often affects adolescents and young adults. It is associated with a unique chromosomal translocation that results in the formation and expression of the fusion gene SS18-SSX, which underlies its pathogenesis. Although SS18-SSX provides a potentially unique therapeutic target, all attempts to neutralise it have been unsuccessful thus far. When complete surgical removal of the tumour fails, therapy is limited to largely ineffective cytotoxic drug regimens. Nevertheless, recent discoveries about the mechanisms of SS18-SSX protein function have provided insight into potential alternative therapeutic strategies. SS18-SSX displays oncogenic activity through protein-protein interactions and participation in chromatin remodelling complexes. This review summarises our current understanding of the function of SS18-SSX and the mechanisms by which it alters the epigenetic landscape of permissive cells to induce transformation and the subsequent development of synovial sarcoma.
Keywords
Cell Transformation, Neoplastic/genetics, Chromatin/genetics, Epigenesis, Genetic, Humans, Neoplasm Proteins/genetics, Proto-Oncogene Proteins/genetics, Repressor Proteins/genetics, Sarcoma, Synovial/genetics, Sarcoma, Synovial/pathology, Translocation, Genetic/genetics
Pubmed
Web of science
Open Access
Yes
Create date
11/12/2018 9:18
Last modification date
20/08/2019 15:05
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