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Targeting the p53 pathway in Ewing Sarcoma

journal contribution
posted on 2018-10-04, 00:00 authored by Paul NeilsenPaul Neilsen, KI Pishas, DF Callen, DM Thomas
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. Copyright © 2011 Paul M. Neilsen et al.

Funding

Category 3 - Industry and Other Research Income

History

Volume

2011

Start Page

1

End Page

17

Number of Pages

17

eISSN

1369-1643

ISSN

1357-714X

Publisher

Hindawi

Peer Reviewed

  • Yes

Open Access

  • Yes

External Author Affiliations

University of Adelaide and Hanson Institute; Ian Potter Foundation Centre for Cancer Genomics and Predictive Medicine

Era Eligible

  • Yes

Journal

Sarcoma

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