The EWS-WT1 gene fusion in desmoplastic small round cell tumor Journal Article
| Authors: | Gerald, W. L.; Haber, D. A. |
| Article Title: | The EWS-WT1 gene fusion in desmoplastic small round cell tumor |
| 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. © 2005 Elsevier Ltd. All rights reserved. |
| Keywords: | unclassified drug; histopathology; review; cancer diagnosis; dna replication; neoplasms; animals; gene; gene targeting; stat3 protein; protein targeting; molecular dynamics; transcription factor; transcription factors; chimera; gene expression regulation; chromosome aberration; myelodysplastic syndrome; transcription regulation; messenger rna; gene identification; gene fusion; oncogene proteins, fusion; protein induction; chromosome translocation; immunophenotyping; wt1 protein; protein family; dna binding; rna binding protein ews; clinical examination; desmoplastic small round cell tumor; rna-binding protein ews; leukemia antigen; wt1 proteins; interleukin 2 receptor beta; chromosomal translocation; platelet derived growth factor a; dsrct; ews-wt1; t cell acute lymphoblastic leukemia associated antigen 1; lrrc15 gene; myeloid leukemia factor 1 gene |
| Journal Title: | Seminars in Cancer Biology |
| Volume: | 15 |
| Issue: | 3 |
| ISSN: | 1044-579X |
| Publisher: | Academic Press Inc., Elsevier Science |
| Date Published: | 2005-06-01 |
| Start Page: | 197 |
| End Page: | 205 |
| Language: | English |
| DOI: | 10.1016/j.semcancer.2005.01.005 |
| PUBMED: | 15826834 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 31" - "Export Date: 24 October 2012" - "CODEN: SECBE" - "Source: Scopus" |
