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Type: Journal article
Title: Targeting the p53 pathway in Ewing Sarcoma
Author: Neilsen, P.
Pishas, K.
Callen, D.
Thomas, D.
Citation: Sarcoma, 2011; 2011:746939-1-746939-17
Publisher: Hindawi Publishing Corporation
Issue Date: 2011
ISSN: 1357-714X
Statement of
Paul M. Neilsen, Kathleen I. Pishas, David F. Callen and David M. Thomas
Abstract: The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downstream signaling pathways and DNA-damage cell cycle checkpoints remain functionally intact in these sarcomas. This paper summarizes recent insights into the functional capabilities and regulation of p53 in Ewing Sarcoma, with a particular focus on the cross-talk between p53 and the EWS-FLI1 gene rearrangement frequently associated with this disease. The development of several activators of p53 is discussed, with recent evidence demonstrating the potential of small molecule p53 activators as a promising systemic therapeutic approach for the treatment of Ewing Sarcomas with wild-type p53.
Description: Extent: 17p.
Rights: Copyright © 2011 Paul M. Neilsen et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
RMID: 0020110962
DOI: 10.1155/2011/746939
Appears in Collections:Medicine publications

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