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Oncogene. 2001 Sep 10;20(40):5755-62.

Fusions of the SYT and SSX genes in synovial sarcoma.

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1
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA. ladanyim@mskcc.org

Abstract

Synovial sarcomas are high grade spindle cell tumors that are divided into two major histologic subtypes, biphasic and monophasic, according to the respective presence or absence of a well-developed glandular epithelial component. They contain in essentially all cases a t(X;18) representing the fusion of SYT (at 18q11) with either SSX1 or SSX2 (both at Xp11). Neither SYT, nor the SSX proteins contain DNA-binding domains. Instead, they appear to be transcriptional regulators whose actions are mediated primarily through protein-protein interactions, with BRM in the case of SYT, and with Polycomb group repressors in the case of SSX. Ongoing work on the SYT-SSX fusion and synovial sarcoma should yield a variety of data of broader biological interest, in areas such as BRM and Polycomb group function and dysfunction, transcriptional targets of SYT-SSX proteins and their native counterparts, differential gene regulation by SYT-SSX1 and SYT-SSX2, control of glandular morphogenesis, among others.

PMID:
11607825
DOI:
10.1038/sj.onc.1204601
[Indexed for MEDLINE]
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