Synovial sarcoma: recent discoveries as a roadmap to new avenues for therapy.

ABSTRACT:

Unlabelled

Oncogenesis in synovial sarcoma is driven by the chromosomal translocation t(X,18; p11,q11), which generates an in-frame fusion of the SWI/SNF subunit SS18 to the C-terminal repression domains of SSX1 or SSX2. Proteomic studies have identified an integral role of SS18-SSX in the SWI/SNF complex, and provide new evidence for mistargeting of polycomb repression in synovial sarcoma. Two recent in vivo studies are highlighted, providing additional support for the importance of WNT signaling in synovial sarcoma: One used a conditional mouse model in which knockout of β-catenin prevents tumor formation, and the other used a small-molecule inhibitor of β-catenin in xenograft models.

Significance

Synovial sarcoma appears to arise from still poorly characterized immature m

SUBMITTER: Nielsen TO 

PROVIDER: S-EPMC4320664 | biostudies-literature | 2015 Feb

REPOSITORIES: biostudies-literature