Towards an understanding of the role of p53 in adrenocortical carcinogenesis.
Towards an understanding of the role of p53 in adrenocortical carcinogenesis.
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DOI：
10.1016/j.mce.2011.09.010
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发表时间：
2012-03-31
影响因子：
4.1
通讯作者：
Malkin, David
中科院分区：
文献类型：
--
作者：
Wasserman, Jonathan D.;Zambetti, Gerard P.;Malkin, David
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.
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