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Andrea Gutierrez Maria, Christina Tatsi, Annabel Berthon, Ludivine Drougat, Nikolaos Settas, Fady Hannah-Shmouni, Jerome Bertherat, Fabio R Faucz, and Constantine A Stratakis

) cortisol-producing adenomas (CPA) causing CS ( Bertherat et al. 2003 , 2009 ). The importance of the role of cAMP-signaling for adrenocortical function and regulation of cortisol secretion was further enhanced by the discovery of cAMP

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Andrew E Greenstein, Mouhammed Amir Habra, Subhagya A Wadekar, and Andreas Grauer

outcomes across multiple oncology indications ( Habra et al. 2019 , Head et al. 2019 ). Cortisol is a potent, abundant, and immunosuppressive endogenous GC and its target, the glucocorticoid receptor (GR), is expressed in nearly every cell in the body

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Christina Wei and Elizabeth C Crowne

other endocrine disorders such as growth hormone deficiency, gonadal failure and hypothyroidism, but there can be a significant impact on morbidity and mortality risk ( Rose et al. 2004 ). Under physiological stress, undiagnosed cortisol deficiency may

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Kiran Nadella, Fabio R Faucz, and Constantine A Stratakis

corticotropin-independent Cushing syndrome ( Kirschner et al. 2000 , Horvath et al. 2010 ). PPNAD is a rare form of bilateral adrenocortical hyperplasia that is genetically distinct from other forms of cortisol-producing pituitary tumors, such as primary

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Fady Hannah-Shmouni, Annabel Berthon, Fabio R Faucz, Juan Medina Briceno, Andrea Gutierrez Maria, Andrew Demidowich, Mirko Peitzsch, Jimmy Masjkur, Fidéline Bonnet-Serrano, Anna Vaczlavik, Jérôme Bertherat, Martin Reincke, Graeme Eisenhofer, and Constantine A Stratakis

( Vassiliadi & Tsagarakis 2019 ); cortisol, its precursor steroids, and occasionally other hormones, such as aldosterone, are aberrantly regulated by non-mutated eutopic and/or ectopic G-protein coupled receptors (GPCRs) ( El Ghorayeb et al. 2015

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Stephanie Espiard, Ludivine Drougat, Nikolaos Settas, Sara Haydar, Kerstin Bathon, Edra London, Isaac Levy, Fabio R Faucz, Davide Calebiro, Jérôme Bertherat, Dong Li, Michael A Levine, and Constantine A Stratakis

non-endocrine disorders ( Kirschner et al. 2000 , Horvath et al. 2010 ). Somatic PRKAR1A mutations are present in isolated primary pigmented nodular adrenocortical disease (PPNAD) and cortisol-producing adenomas (CPA) causing Cushing syndrome

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E Louiset, K Isvi, J M Gasc, C Duparc, B Cauliez, A Laquerrière, J M Kuhn, and H Lefebvre

Introduction We have previously shown that, in the normal human adrenal gland, serotonin (5-hydroxy tryptamine; 5-HT), released by perivascular mast cells, stimulates aldosterone and cortisol production through a paracrine mechanism involving 5-HT 4

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Peter M van Koetsveld, Giovanni Vitale, Richard A Feelders, Marlijn Waaijers, Diana M Sprij-Mooij, Ronald R de Krijger, Ernst-Jan M Speel, Johannes Hofland, Steven W J Lamberts, Wouter W de Herder, and Leo J Hofland

in primary cultures of ACC. In this study, we evaluated the antitumor activity of type 1 IFNs, with or without mitotane, in primary ACC cultures. The effects of both drugs on cortisol production were studied and a comparison was made with the effects

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Maria Cristina De Martino, Peter M van Koetsveld, Richard A Feelders, Diana Sprij-Mooij, Marlijn Waaijers, Steven W J Lamberts, Wouter W de Herder, Annamaria Colao, Rosario Pivonello, and Leo J Hofland

autocrine loop in the effects of mTOR inhibitors; and iv) evaluate the effect of mTOR inhibitors on cortisol secretion. Materials and methods Study methodology In this study, we characterized the expression of the mTOR and IGF2 in three different human ACC

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Dimitra A Vassiliadi and Stylianos Tsagarakis

secretion that compensates for enzymatic defects of cortisol synthesis as in patients with congenital adrenal hyperplasia (CAH). Kirschner et al. (1964) described an unusual form of Cushing’s syndrome (CS), characterized by the constellation of long