Biology of EWS/ETS fusions in Ewing's family tumors

被引：276

作者：

Arvand, A

Denny, CT ^{[1
]}

机构：

[1] Univ Calif Los Angeles, Inst Mol Biol, Gwynee Hazen Cherry Mem Lab, Los Angeles, CA 90024 USA

[2] Univ Calif Los Angeles, Jonsson Comprehens Ctr, Gwynee Hazen Cherry Mem Lab, Los Angeles, CA 90024 USA

[3] Univ Calif Los Angeles, Dept Pathol & Lab Med, Gwynee Hazen Cherry Mem Lab, Los Angeles, CA 90024 USA

[4] Univ Calif Los Angeles, Dept Pediat, Gwynee Hazen Cherry Mem Lab, Los Angeles, CA 90024 USA

来源：

ONCOGENE | 2001年 / 20卷 / 40期

关键词：

EWS/ETS; Ewing's family tumors; aberrant transcription factors;

D O I：

10.1038/sj.onc.1204598

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Tumor-associated chromosomal translocations lead to the formation of chimeric fusions between the EWS gene and one of five different ETS transcription factors in Ewing's family tumors (EFTs). The resultant EWS/ETS proteins promote oncogenesis in a dominant fashion in model systems and are necessary for continued growth of EFT cell lines. EWS belongs to a family of genes that encode proteins that may serve as adapters between the RNA polymerase II complex and RNA splicing factors. EWS/ETS fusions have biochemical characteristics of aberrant transcription factors and appear to promote abnormal cellular growth by transcriptionally modulating a network of target genes. Early evidence suggests that EWS/ETS proteins may also impact gene expression through alteration in RNA processing. Elucidation of EWS/ETS target gene networks in the context of other signaling pathways will hopefully lead to biology based therapeutic strategies for EFT.

引用

页码：5747 / 5754

页数：8

共 111 条

[1]

Athanasiou M, 1996, CELL GROWTH DIFFER, V7, P1525

[2] Identification of a novel fusion gene involving hTAFII68 and CHN from a t(9;17)(q22;q11.2) translocation in an extraskeletal myxoid chondrosarcoma [J].