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      Orphan receptors COUP-TF and DAX-1 as targets in disordered CYP17 expression in adrenocortical tumors.

      Endocrine research

      Adrenal Cortex Neoplasms, genetics, COUP Transcription Factors, DAX-1 Orphan Nuclear Receptor, DNA-Binding Proteins, metabolism, Fushi Tarazu Transcription Factors, Gene Expression, physiology, Homeodomain Proteins, Humans, RNA, Messenger, Receptors, Cytoplasmic and Nuclear, Receptors, Retinoic Acid, Receptors, Steroid, Repressor Proteins, Steroid 17-alpha-Hydroxylase, Steroidogenic Factor 1, Transcription Factors

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          CYP17 gene transcription is activated by SF-1 binding to a cyclic AMP-responsive sequence within the promoter region of the gene, and its transcription is inhibited by COUP-TF binding to the sequence. Another orphan receptor, DAX-1, is shown to act as a suppressor of SF-1-mediated transcription. We examined the expression level of these orphan receptors in adrenocortical tumors and compared the results with CYP17 expression. CYP17 was highly expressed in cortisol-producing adenomas, whereas COUP-TF and DAX-1 expression levels were very low. In deoxycorticosterone-producing adenomas, on the other hand, CYP17 expression was extremely low, whereas DAX-1 was highly expressed and SF-1 expression was slightly decreased. In conclusion, the reciprocal expression of CYP17 and the transcriptional repressors COUP-TF and DAX-1 indicates that these orphan receptors have a pathophysiologic role in the excessive hormone production in cortisol- and deoxycorticosterone-producing adrenocortical tumors.

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