SS18 (synovial sarcoma translocation, chromosome 18)
2006-05-01 Mamoru Ouchida   AffiliationDepartment of Molecular Genetics, Graduate School of Medicine, Dentistry, Pharmaceutical Sciences, Okayama University. Shikata-cho 2-5-1, Okayama 700-8558, Japan
DNA/RNA
Note
Member of the SS18 family. SYT, one of the alternative gene names, has continued to be used in the literature for this gene.
Description
11 exons, spaning approximately 75 kb of genomic DNA in the telomere-to-centromere orientation on chromosome 18q11.2 The promoter region lacks CAAT and TATA boxes but contains CpG islands, suggesting that SS18 is a housekeeping gene.
Transcription
Exon 8 is spliced out with different ratio in the various tissues by alternative splicing.
Pseudogene
SS18L2 (3p21)
Proteins
Description
418 amino acids. 2 domains; the SHN domain (the SYT N-terminal homology domain) that is found in proteins from a wide variety of species ranging from plants to human, and the QPGY domain at the C-terminal part, rich in glutamine, proline, glycine and tyrosine. Four putative src-homology binding domains and two annexin-like direct repeats were exhibited in the SS18 protein. SS18 protein binds to p300, hBRM, AF10 and SIN3A proteins. The QPGY domain activates transcription when coupled to a DNA-binding domain.
Expression
Ubiquitous
Localisation
Nuclear
Function
Transcriptional coactivator
Implicated in
Entity name
Prognosis
Cytogenetics
Hybrid gene
SYT-SSX1, SYT-SSX2, SYT-SSX4; the exon 10 of the SS18 (SYT) gene is fused to exon 6 of the SSX genes.
Fusion protein
The last 8 amino acid residues of the SS18 (SYT) protein are replaced by 78 amino acids from the C-terminal part of SSX proteins.
Breakpoints

Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 7951320 | 1994 | Identification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcoma. | Clark J et al |
| 11030627 | 2000 | p300 interacts with the nuclear proto-oncoprotein SYT as part of the active control of cell adhesion. | Eid JE et al |
| 15467731 | 2004 | SYT, a partner of SYT-SSX oncoprotein in synovial sarcomas, interacts with mSin3A, a component of histone deacetylase complex. | Ito T et al |
| 9428816 | 1998 | SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. | Kawai A et al |
| 12963121 | 2003 | Splicing isoform of SYT-SSX fusion protein accelerates transcriptional activity and cell proliferation. | Morimoto Y et al |
| 11064357 | 2000 | A reverse transcriptase-polymerase chain reaction assay in the diagnosis of soft tissue sarcomas. | Naito N et al |
| 10359553 | 1999 | A novel fusion gene, SYT-SSX4, in synovial sarcoma. | Skytting B et al |
| 10072425 | 1999 | Functional domains of the SYT and SYT-SSX synovial sarcoma translocation proteins and co-localization with the SNF protein BRM in the nucleus. | Thaete C et al |
| 11435705 | 2001 | Mapping and characterization of the mouse and human SS18 genes, two human SS18-like genes and a mouse Ss18 pseudogene. | de Bruijn DR et al |
Other Information
Locus ID:
NCBI: 6760
MIM: 600192
HGNC: 11340
Ensembl: ENSG00000141380
Variants:
dbSNP: 6760
ClinVar: 6760
TCGA: ENSG00000141380
COSMIC: SS18
RNA/Proteins
Expression (GTEx)
Pathways
| Pathway | Source | External ID |
|---|---|---|
| Transcriptional misregulation in cancer | KEGG | ko05202 |
| Transcriptional misregulation in cancer | KEGG | hsa05202 |
Protein levels (Protein atlas)
References
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 36221318 | 2023 | Microcribriform Adenocarcinoma of Salivary Glands: A Unique Tumor Entity Characterized by an SS18::ZBTB7A Fusion. | 4 |
| 36591871 | 2023 | SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients. | 1 |
| 36221318 | 2023 | Microcribriform Adenocarcinoma of Salivary Glands: A Unique Tumor Entity Characterized by an SS18::ZBTB7A Fusion. | 4 |
| 36591871 | 2023 | SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients. | 1 |
| 34091760 | 2021 | Correlating SS18-SSX immunohistochemistry (IHC) with SS18 fluorescent in situ hybridization (FISH) in synovial sarcomas: a study of 36 cases. | 7 |
| 34215745 | 2021 | SS18 regulates pluripotent-somatic transition through phase separation. | 12 |
| 34091760 | 2021 | Correlating SS18-SSX immunohistochemistry (IHC) with SS18 fluorescent in situ hybridization (FISH) in synovial sarcomas: a study of 36 cases. | 7 |
| 34215745 | 2021 | SS18 regulates pluripotent-somatic transition through phase separation. | 12 |
| 32747783 | 2020 | The nucleosome acidic patch and H2A ubiquitination underlie mSWI/SNF recruitment in synovial sarcoma. | 20 |
| 32747783 | 2020 | The nucleosome acidic patch and H2A ubiquitination underlie mSWI/SNF recruitment in synovial sarcoma. | 20 |
| 30814111 | 2019 | SS18-SSX-Dependent YAP/TAZ Signaling in Synovial Sarcoma. | 22 |
| 31094920 | 2019 | Microsecretory Adenocarcinoma: A Novel Salivary Gland Tumor Characterized by a Recurrent MEF2C-SS18 Fusion. | 27 |
| 30814111 | 2019 | SS18-SSX-Dependent YAP/TAZ Signaling in Synovial Sarcoma. | 22 |
| 31094920 | 2019 | Microsecretory Adenocarcinoma: A Novel Salivary Gland Tumor Characterized by a Recurrent MEF2C-SS18 Fusion. | 27 |
| 29502955 | 2018 | The SS18-SSX Oncoprotein Hijacks KDM2B-PRC1.1 to Drive Synovial Sarcoma. | 57 |
Citation
Mamoru Ouchida
SS18 (synovial sarcoma translocation, chromosome 18)
Atlas Genet Cytogenet Oncol Haematol. 2006-05-01
Online version: http://atlasgeneticsoncology.org/gene/84/ss18
