Fusions of the SYT and SSX genes in synovial sarcoma

Oncogene. 2001 Sep 10;20(40):5755-62. doi: 10.1038/sj.onc.1204601.


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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Cell Differentiation
  • Cell Division
  • Cell Nucleus / metabolism
  • Humans
  • Oncogene Proteins, Fusion / chemistry*
  • Oncogene Proteins, Fusion / genetics*
  • Oncogene Proteins, Fusion / metabolism*
  • Phenotype
  • Protein Structure, Tertiary
  • Sarcoma, Synovial / genetics*
  • Sarcoma, Synovial / metabolism*
  • Signal Transduction
  • Translocation, Genetic


  • Oncogene Proteins, Fusion
  • SYT-SSX fusion protein