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Fusions of the SYT and SSX genes in synovial sarcoma

Oncogene volume 20, pages 5755–5762 (2001)Cite this article

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.

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Acknowledgements

ML is supported by grant PO1 CA47179 from the National Institute of Health.

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

    Marc Ladanyi

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Correspondence to Marc Ladanyi.

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Ladanyi, M. Fusions of the SYT and SSX genes in synovial sarcoma. Oncogene 20, 5755–5762 (2001). https://doi.org/10.1038/sj.onc.1204601

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