title: Fusions of the SYT and SSX genes in synovial sarcoma.
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.
authors.affiliation: Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA. ladanyim@mskcc.org
authors.forename: M
authors.initials: M
authors.lastname: Ladanyi
mesh_terms: D002454:Cell Differentiation; D002455:Cell Division; D002467:Cell Nucleus / Q000378:metabolism; D006801:Humans; D015514:Oncogene Proteins, Fusion / Q000737:chemistry* / Q000235:genetics* / Q000378:metabolism*; D010641:Phenotype; D017434:Protein Structure, Tertiary; D013584:Sarcoma, Synovial / Q000235:genetics* / Q000378:metabolism*; D015398:Signal Transduction; D014178:Translocation, Genetic
chemical_list: D015514:Oncogene Proteins, Fusion; C116512:SYT-SSX fusion protein