PubMed · 14844698
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E SCHWARZ-TIENE. 1951. [Adrenal cortex function in pediatric pathology].. https://pubmed.ncbi.nlm.nih.gov/14844698/
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Beacon, a hypothalamic peptide involved in the regulation of food intake, has been recently shown to be expressed in the adrenal cortex, and to inhibit its secretion and growth. To further characterize the role of beacon in the control of adrenal growth, we investigated the level of beacon gene expression in the regenerating rat adrenal cortex. Conventional reverse transcription-polymerase chain reaction (PCR) and immunocytochemistry demonstrated the expression of beacon mRNA and protein in the adrenals at both days 5 and 8 of regeneration after enucleation and contralateral adrenalectomy. Semiquantitative real time-PCR revealed a net down-regulation of beacon mRNA in the regenerating glands, as compared to the intact adrenal cortex of sham-operated animals. Beacon gene expression was higher at day 8 than at day 5 of regeneration. Mitotic index, as assayed by the stachmokinetic method with vincristin, was negligible in the intact adrenal, but greatly elevated in regenerating gland, with a higher index found at day 5 than at day 8 after surgery. Taken together our findings indicate that the level of beacon gene expression is inversely correlated with the proliferative activity of adrenocortical cells, and suggest that beacon might act as an endogenous inhibitor of adrenocortical growth in the rat.
A variety of transcription factors including Wilms tumor gene (Wt-1), steroidogenic factor 1 (Sf-1), dosage-sensitive sex reversal, adrenal hypoplasia congenita on the X-chromosome, Gene 1 (Dax-1), and pre-B-cell transcription factor 1 (Pbx1) have been defined as necessary for regular adrenocortical development. However, the role of Pbx1 for adrenal growth and function in the adult organism together with the molecular relationship between Pbx1 and these other transcription factors have not been characterized. We demonstrate that Pbx haploinsufficiency (Pbx1(+/-)) in mice is accompanied by a significant lower adrenal weight in adult animals compared with wild-type controls. Accordingly, baseline proliferating cell nuclear antigen levels are lower in Pbx1(+/-) mice, and unilateral adrenalectomy results in impaired contralateral compensatory adrenal growth, indicating a lower proliferative potential in the context of Pbx1 haploinsufficiency. In accordance with the key role of IGFs in adrenocortical proliferation and development, real-time RT-PCR demonstrates significant lower expression levels of the IGF-I receptor, and up-regulation of IGF binding protein-2. Functionally, Pbx1(+/-) mice display a blunted corticosterone response after ACTH stimulation coincident with lower adrenal expression of the ACTH receptor (melanocortin 2 receptor, Mc2-r). Mechanistically, in vitro studies reveal that Pbx1 and Sf-1 synergistically stimulates Mc2-r promoter activity. Moreover, Sf-1 directly activates the Pbx1 promoter activity in vitro and in vivo. Taken together, these studies provide evidence for a role of Pbx1 in the maintenance of a functional adrenal cortex mediated by synergistic actions of Pbx1 and Sf-1 in the transcriptional regulation of the critical effector of adrenocortical differentiation, the ACTH receptor.
Ectopic adrenocortical tissues (EAT) are rare pathologies in children. They are frequently encountered in the inguinal region incidentally during surgical procedures but they are also classically known to be located from diaphragm to pelvis. EAT were investigated during inguinal procedures including inguinal hernia, hydrocele, hydrocele of cord and undescended testis (UDT) in 1,069 patients, 159 girls, 910 boys, from April 1997 until April 2006. All these nodules were confirmed to be adrenocortical tissues after histopathological examinations. Statistical analysis was done with Fisher's exact test. EAT were noted on the plexus pampiniformis external to processus vaginalis of 22 male patients (1.73%) during inguinal explorations. This figure was 1.63% for inguinal explorations of inguinal hernia, hydrocele and hydrocele of cord and 5.1% for UDT. This difference was statistically significant (P<0.0001). There were no EAT detected during inguinal exploration of female patients. EAT are uncommon pathologies seen during inguinoscrotal explorations. Incidence of EAT is significantly increased with UDT. This increase may be explained with the embryological events that take place during adrenal and gonadal development. The relatively low observation of EAT in girls may be due to the localization of gonads and association of EAT with gonadal position. Adrenal rests in inguinal region may suggest an association with descent of gonads.