EWS–ETS oncoproteins: The linchpins of Ewing tumors

Ewing tumors, which comprise Ewing's sarcoma and peripheral primitive neuroectodermal tumors, are highly aggressive and mostly affect children and adolescents. Their molecular signature is a chromosomal translocation leading to the generation of EWS–ETS (or very rarely FUS–ETS) fusion proteins...

Full description

Saved in:
Bibliographic Details
Published in:Gene 2005-12, Vol.363, p.1-14
Main Author: Janknecht, Ralf
Format: Article
Language:English
Subjects:
Cancer
Chromosomes, Human, Pair 16
Chromosomes, Human, Pair 21
Ewing's sarcoma protein
FUS
Gene transcription
Humans
Proto-Oncogene Proteins c-ets - genetics
Proto-Oncogene Proteins c-ets - physiology
RNA splicing
RNA-Binding Protein EWS - genetics
RNA-Binding Protein EWS - physiology
Sarcoma, Ewing - genetics
Sarcoma, Ewing - physiopathology
Translocation, Genetic
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Ewing tumors, which comprise Ewing's sarcoma and peripheral primitive neuroectodermal tumors, are highly aggressive and mostly affect children and adolescents. Their molecular signature is a chromosomal translocation leading to the generation of EWS–ETS (or very rarely FUS–ETS) fusion proteins that are capable of transforming cells. These oncoproteins act as aberrant transcription factors due to the fusion of an ETS DNA binding domain to a highly potent EWS (or FUS) transactivation domain. Accordingly, many EWS–ETS target genes have been identified whose dysregulation could contribute to the development of tumor formation. Furthermore, EWS–ETS oncoproteins may impact on RNA splicing or affect other proteins through disturbing their ability to form functional complexes. The molecular knowledge gained so far from studying EWS–ETS oncoproteins has not only broadened our understanding of Ewing tumors but also improved the diagnosis of these highly undifferentiated tumors. In addition, several potential prognostic markers have been uncovered and novel therapies are suggested that may improve the still dismal survival rate of Ewing tumor patients.
ISSN:0378-1119
1879-0038
DOI:10.1016/j.gene.2005.08.007