Gerald WL, Haber DA.. The EWS-WT1 gene fusion in desmoplastic small round cell tumor. Sem Cancer Biol 15: 197-205

ArticleinSeminars in Cancer Biology 15(3):197-205 · July 2005with14 Reads
Impact Factor: 9.33 · DOI: 10.1016/j.semcancer.2005.01.005 · Source: PubMed

    Abstract

    Desmoplastic small round cell tumor (DSRCT) is a poorly understood neoplasm with distinctive clinical, histologic and immunophenotypic features. It is associated with a novel, specific chromosomal abnormality, t(11;22)(p13;q12) that fuses EWS with WT1 leading to production of a chimeric protein with transcriptional regulatory activity. This chimeric transcription factor has unique DNA-binding properties and regulates expression of specific target genes. Several of these have been identified and their biological role characterized. The dysregulated expression of EWS-WT1 targets contribute to the malignant phenotype of DSRCT and provide valuable insight regarding the molecular mechanisms underlying the development and progression of this distinct translocation associated tumor.