Journal
MOLECULAR AND CELLULAR ENDOCRINOLOGY
Volume 351, Issue 1, Pages 101-110Publisher
ELSEVIER IRELAND LTD
DOI: 10.1016/j.mce.2011.09.010
Keywords
p53; Adrenocortical carcinoma; Pediatric; ACC
Categories
Funding
- National Institutes of Health [CA-21765, GM-083159]
- Canadian Pediatric Endocrine Group
- Pediatric Endocrine Society
- American Lebanese Syrian Associated Charities
- Canadian Cancer Society Research Institute [18435]
- Canadian Institutes of Health Research [MOP-97834]
- SickKids Foundation
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Adrenocortical carcinoma (ACC) is recognized to be a component tumor of the Li Fraumeni Syndrome (LFS), a familial cancer predisposition resulting from germline mutations in the p53 tumor-suppressor. p53 activity is tightly regulated by multiple post-translational mechanisms, disruption of which may lead to tumorigenesis. ACC is present in disproportionately high rates among p53-mutation carriers, suggesting tissue-specific manifestations of p53 deficiency. Additionally, p53-associated ACC demonstrates a strong predominance in infants and children. Several of the p53 alleles associated with pediatric ACC, however, retain significant wild-type activity and demonstrate incomplete penetrance, a finding distinct from other LFS-component tumors. In this review, we discuss the relationship between p53 and adrenocortical carcinogenesis, with specific focus on disease-specific alleles, tumorigenesis in the context of adrenal development and potential therapeutic approaches to p53-associated ACC. (C) 2011 Elsevier Ireland Ltd. All rights
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