Document Detail


Towards an understanding of the role of p53 in adrenocortical carcinogenesis.
MedLine Citation:
PMID:  21930187     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Jonathan D Wasserman; Gerard P Zambetti; David Malkin
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2011-09-10
Journal Detail:
Title:  Molecular and cellular endocrinology     Volume:  351     ISSN:  1872-8057     ISO Abbreviation:  Mol. Cell. Endocrinol.     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-02-20     Completed Date:  2012-06-11     Revised Date:  2013-06-27    
Medline Journal Info:
Nlm Unique ID:  7500844     Medline TA:  Mol Cell Endocrinol     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  101-10     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.
Affiliation:
Division of Endocrinology, Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8. Jonathan.Wasserman@sickkids.ca
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adrenal Cortex Neoplasms* / genetics,  metabolism,  pathology,  therapy
Adrenocortical Carcinoma* / genetics,  metabolism,  pathology,  therapy
Alleles*
Animals
Cell Transformation, Neoplastic* / genetics,  metabolism,  pathology
Child
Child, Preschool
Humans
Infant
Li-Fraumeni Syndrome / genetics,  metabolism,  pathology,  therapy
Organ Specificity
Penetrance*
Tumor Suppressor Protein p53* / genetics,  metabolism
Grant Support
ID/Acronym/Agency:
CA-21765/CA/NCI NIH HHS; GM-083159/GM/NIGMS NIH HHS; MOP-97834//Canadian Institutes of Health Research; R01 CA063230-09/CA/NCI NIH HHS
Chemical
Reg. No./Substance:
0/TP53 protein, human; 0/Tumor Suppressor Protein p53
Comments/Corrections

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