Document Detail


ACTH-receptor expression, regulation and role in adrenocortial tumor formation.
MedLine Citation:
PMID:  11248736     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The regulation of the ACTH-receptor gene is unique in that it is up-regulated by its own ligand, ACTH. Ligand-induced up-regulation of ACTH-receptor expression may be an important adaptive process directed towards optimizing adrenal responsiveness to ACTH in the context of physiological stress and the maintenance of metabolic homeostasis in which the adrenals play a pivotal role. Whereas enhancement by ligand-induced up-regulation permits a more efficient and rapid glucocorticoid response, negative feedback regulation of glucocorticoids in the hypothalamus and pituitary inhibits ACTH secretion and allows a balanced adrenal response to stress. Since the cloning of the promoter region of the ACTH receptor, considerable progress in the understanding of the regulatory processes has been made. The effects of ACTH on ACTH-receptor expression is dependent on cAMP, probably mediated through AP-1. The profound effect of three SF-1-binding sites in the ACTH-receptor promoter was demonstrated by deletion experiments. Conversely, ACTH-receptor expression can be suppressed by adrenal-specific transcription factors,like DAX-1. Despite an extensive search, no activating ACTH-receptor mutations have been found in adrenal tumors,excluding the ACTH receptor as a relevant oncogene in adrenal tumorigenesis. However, the ACTH receptor may act as a differentiation factor as suggested by LOH in adrenal carcinomas with an undifferentiated tumor type. In benign adrenal tumors, a strong correlation between ACTH-receptor expression and expression of P450 steroidogenic enzymes is evident. This close regulative relationship is lost in adrenal carcinoma, probably as a result of tumor dedifferentiation. Down-regulation of ACTH-receptor expression in normal and neoplastic tissue can be achieved by adrenostatic compounds such as aminoglutethimide and metyrapone.
Authors:
F Beuschlein; M Fassnacht; A Klink; B Allolio; M Reincke
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  European journal of endocrinology / European Federation of Endocrine Societies     Volume:  144     ISSN:  0804-4643     ISO Abbreviation:  Eur. J. Endocrinol.     Publication Date:  2001 Mar 
Date Detail:
Created Date:  2001-03-15     Completed Date:  2001-04-26     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  9423848     Medline TA:  Eur J Endocrinol     Country:  England    
Other Details:
Languages:  eng     Pagination:  199-206     Citation Subset:  IM    
Affiliation:
Schwerpunkt Endokrinologie, Abteilung Innere Medizin II, Klinikum der Albert-Ludwigs-Universitat, Freiburg, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adrenal Cortex Neoplasms / genetics,  metabolism*,  pathology*
Animals
Exons / genetics
Gene Expression Regulation* / drug effects
Humans
Introns / genetics
Loss of Heterozygosity
Point Mutation / genetics
Promoter Regions, Genetic / genetics
RNA, Messenger / genetics,  metabolism
Receptors, Corticotropin / genetics,  metabolism*
Chemical
Reg. No./Substance:
0/RNA, Messenger; 0/Receptors, Corticotropin

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