http://purl.uniprot.org/citations/7862145 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/7862145 | http://www.w3.org/2000/01/rdf-schema#comment | "Alveolar rhabdomyosarcomas are pediatric solid tumors with a hallmark cytogenetic abnormality: translocation of chromosomes 2 and 13 [t(2;13) (q35;q14)]. The genes on each chromosome involved in this translocation have been identified as the transcription factor-encoding genes PAX3 and FKHR. The NH2-terminal paired box and homeodomain DNA-binding domains of PAX3 are fused in frame to COOH-terminal regions of the chromosome 13-derived FKHR gene, a novel member of the forkhead DNA-binding domain family. To determine the role of the fusion protein in transcriptional regulation and oncogenesis, we identified the PAX3-FKHR fusion protein and characterized its function(s) as a transcription factor relative to wild-type PAX3. Antisera specific to PAX3 and FKHR were developed and used to examine PAX3 and PAX3-FKHR expression in tumor cell lines. Sequential immunoprecipitations with anti-PAX3 and anti-FKHR sera demonstrated expression of a 97-kDa PAX3-FKHR fusion protein in the t(2;13)-positive rhabdomyosarcoma Rh30 cell line and verified that a single polypeptide contains epitopes derived from each protein. The PAX3-FKHR protein was localized to the nucleus in Rh30 cells, as was wild-type PAX3, in t(2;13)-negative A673 cells. In gel shift assays using a canonical PAX binding site (e5 sequence), we found that DNA binding of PAX3-FKHR was significantly impaired relative to that of PAX3 despite the two proteins having identical PAX DNA-binding domains. However, the PAX3-FKHR fusion protein was a much more potent transcriptional activator than PAX3 as determined by transient cotransfection assays using e5-CAT reporter plasmids. The PAX3-FKHR protein may function as an oncogenic transcription factor by enhanced activation of normal PAX3 target genes."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.org/dc/terms/identifier | "doi:10.1128/mcb.15.3.1522"xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Mukhopadhyay S."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Fredericks W.J."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Rovera G."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Galili N."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Barr F.G."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Rauscher F.J."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/author | "Bennicelli J."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/date | "1995"xsd:gYear |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/name | "Mol Cell Biol"xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/pages | "1522-1535"xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/title | "The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3."xsd:string |
http://purl.uniprot.org/citations/7862145 | http://purl.uniprot.org/core/volume | "15"xsd:string |
http://purl.uniprot.org/citations/7862145 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/7862145 |
http://purl.uniprot.org/citations/7862145 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/7862145 |
http://purl.uniprot.org/uniprot/Q12778#attribution-5C67A43AAAB27DC670E4BEB66230EAE3 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/7862145 |