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S18 is a protein that has been shown to be a part of the SWI/SNF complex. The SS18-SSX fusion
proteins are a result of in-frame fusions that fuse the SS18 gene on chromosome 18 with X
chromosome genes SSX1, SSX2, and to a lesser extent SSX4. Human synovial sarcoma (SS) accounts
for 8-10% of all soft tissue malignancies and 95% of these malignancies express the recurrent
translocation of the SS18 gene on chromosome 18. The N-terminal SNH domain (SYT N-terminal
homology domain) of the SS18 protein interacts with SWI/SNF chromatin remodeling complexes via
the N terminal region of BRM and BRG1 subunits. Studies of the SS18-SSX fusion in SS suggest that
endogenous SS18 competes with the mutant SS18-SSX fusion for occupancy in the SWI/SNF complexes
resulting in the displacement of SNF5 (BAF47) subunit. Displacement of the SNF5 subunit results in
altered function of the SWI/SNF complex that leads to deregulated expression of genes such as Sox2
in SS.
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