{"database":"GEO","file_versions":[{"headers":{"Content-Type":["application/json"]},"body":{"files":{"Other":["ftp://ftp.ncbi.nlm.nih.gov/geo/series/GSE303nnn/GSE303259/"]},"type":"primary"},"statusCode":"OK","statusCodeValue":200}],"scores":null,"additional":{"omics_type":["Transcriptomics"],"species":["Mus musculus"],"gds_type":["Expression profiling by high throughput sequencing"],"full_dataset_link":["https://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE303259"],"repository":["GEO"],"entry_type":["GSE"],"additional_accession":[]},"is_claimable":false,"name":"SRF fusion oncogenes encode constitutively activated chimeric transcription factors in myoid soft tissue tumors [C2C12]","description":"SRF fusion genes result from chromosomal translocations in muscle-related soft tissue tumors, including perivascular tumors, myofibroma and rhabdomyosarcoma. SRF encodes the Serum Response Factor, a well-characterized transcription factor that dimerizes and associates with the cofactors TCF or MRTF to regulate muscle development. Here, we characterized four SRF fusion genes: SRF::RELA, SRF::FOXO1, SRF::ICA1L and SRF::PDGFRB. All fusions proteins were nuclear and dimerized through the SRF MADS box. SRF::RELA, SRF::FOXO1, and SRF::ICA1L acted as constitutively active transcription factors, independently of canonical cofactors. Their activity depended on the DNA-binding MADS box of SRF and the transactivation domain of the partner protein. We identified a cryptic transactivation domain within cytosolic ICA1L. These three SRF fusions promoted mesenchymal cell proliferation and upregulated muscle-related genes. They are representative of 98% of the identified SRF fusion cases. In contrast, SRF::PDGFRB activity relied on the tyrosine kinase domain and was sensitive to imatinib. SRF::PDGFRB acted as an oncogene in Ba/F3 cells, activated STAT1 signaling, and induced an interferon-like inflammatory response, in line with the inflammatory myofibroblastic tumor, in which that fusion was identified. In conclusion, SRF fusions constitute a novel family of oncogenes supporting cell proliferation and expression of muscle differentiation markers in myoid soft tissue tumors.","dates":{"publication":"2026/07/18"},"accession":"GSE303259","cross_references":{"GSM":["GSM9122051","GSM9122040","GSM9122050","GSM9122053","GSM9122042","GSM9122041","GSM9122052","GSM9122044","GSM9122043","GSM9122046","GSM9122045","GSM9122048","GSM9122037","GSM9122047","GSM9122039","GSM9122038","GSM9122049"],"GPL":["24247"],"GSE":["303259"],"taxon":["Mus musculus"]}}