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P Nguyen Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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S Khurana Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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H Peltsch Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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J Grandbois Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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J Eibl Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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J Crispo Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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D Ansell Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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T C Tai Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada
Medical Sciences Division, Departments of Biology, Biomolecular Sciences Program, Northern Ontario School of Medicine, Sudbury, Ontario, Canada

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& Sennlaub 2005 , Ligi et al . 2010 ), as well as altered activity of the hypothalamic–pituitary–adrenal (HPA) ( Bouret 2009 , Boyne et al . 2009 ), and the sympatho-adrenal (SA) axes ( Young 2002 , Johansson et al . 2007 ). Further, studies have

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F McManus
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R Fraser
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E Davies
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J M C Connell Institute of Cardiovascular and Medical Sciences, College of Medicine, University of Glasgow, Glasgow Cardiovascular Research Centre, 126 University Place, Glasgow G12 8TA, UK

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E M Freel
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series of rare monogenic disorders of corticosteroid synthesis (congenital adrenal hypertrophy (CAH)), where abnormal levels of intermediate compounds can be both pathogenic and diagnostic ( Miller & Auchus 2011 ). Importantly, qualitatively similar but

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Kelly De Sousa INSERM, UMRS_970, Paris Cardiovascular Research Center, Paris, France
Université Paris Descartes, Sorbonne Paris Cité, Paris, France

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Alaa B Abdellatif INSERM, UMRS_970, Paris Cardiovascular Research Center, Paris, France
Université Paris Descartes, Sorbonne Paris Cité, Paris, France

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Rami M El Zein INSERM, UMRS_970, Paris Cardiovascular Research Center, Paris, France
Université Paris Descartes, Sorbonne Paris Cité, Paris, France

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Maria-Christina Zennaro INSERM, UMRS_970, Paris Cardiovascular Research Center, Paris, France
Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Génétique, Paris, France

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Introduction Primary aldosteronism (PA) is due to excessive and autonomous aldosterone production by the adrenal cortex. It is the most frequent form of secondary hypertension with an estimated prevalence up to 10% in referred patients, 4% in

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Simon Travers Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
Service de Génétique Moléculaire, Pharmacogénétique et Hormonologie, Hôpital de Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France

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Laetitia Martinerie Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
Service d’Endocrinologie Pédiatrique, Hôpital Robert Debré, Assistance Publique Hôpitaux de Paris, Paris, France
Université de Paris, Faculté de Santé, UFR de Médecine, Paris, France
PremUp Fondation, Paris, France

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Qiong-Yao Xue Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
Service de Génétique Moléculaire, Pharmacogénétique et Hormonologie, Hôpital de Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France

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Julie Perrot Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France

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Say Viengchareun Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France

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Kathleen M Caron Department of Cell Biology and Physiology, School of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA

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Elizabeth S Blakeney Department of Cell Biology and Physiology, School of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA

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Pascal Boileau Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
Department of Neonatal Pediatrics, Centre Hospitalier Intercommunal de Poissy-Saint-Germain, Poissy, France
UFR des Sciences de la Santé, Simone Veil. Université Versailles St-Quentin en Yvelines, Montigny le Bretonneux, France

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Marc Lombès Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
PremUp Fondation, Paris, France

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Eric Pussard Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
Service de Génétique Moléculaire, Pharmacogénétique et Hormonologie, Hôpital de Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France

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Introduction Corticosteroid hormones are synthesized in the adrenal cortex, an endocrine gland that produces mineralocorticoids in the zona glomerulosa , glucocorticoids in the zona fasciculata, and adrenal androgens in the zona

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Ronald J van der Sluis Division of Biopharmaceutics, Cluster BioTherapeutics, Gorlaeus Laboratories, Leiden Academic Centre for Drug Research, Einsteinweg 55, 2333 CC Leiden, The Netherlands

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Miranda Van Eck Division of Biopharmaceutics, Cluster BioTherapeutics, Gorlaeus Laboratories, Leiden Academic Centre for Drug Research, Einsteinweg 55, 2333 CC Leiden, The Netherlands

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Menno Hoekstra Division of Biopharmaceutics, Cluster BioTherapeutics, Gorlaeus Laboratories, Leiden Academic Centre for Drug Research, Einsteinweg 55, 2333 CC Leiden, The Netherlands

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Introduction Cholesterol is the sole precursor for the synthesis of steroid hormones including adrenal-derived glucocorticoids, i.e., cortisol in man and corticosterone in rodents. The cholesterol used for steroidogenesis can theoretically be

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Ryo Morimoto
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Fumitoshi Satoh
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Osamu Murakami
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Takuo Hirose Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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Kazuhito Totsune Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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Yutaka Imai Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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Yoichi Arai Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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Takashi Suzuki Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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Hironobu Sasano Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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Sadayoshi Ito
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Kazuhiro Takahashi Division of Nephrology, Department of Clinical Pharmacology and Therapeutics, Department of Urology, Department of Pathology and Histotechnology, Department of Pathology, Department of Endocrinology and Applied Medical Science, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi 980-8574, Japan

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al . 2004 ). It is well known that adrenal medulla and pheochromocytomas express various neuropeptides and vasoactive peptides, such as neuropeptide Y (NPY) and AM, and their receptors ( Hinson et al . 2000 , Spinazzi et al . 2005 ). Moreover

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Chen-Che Jeff Huang Department of Anatomy, Physiology and Pharmacology, College of Veterinary Medicine, Auburn University, Auburn, Alabama, USA

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Yuan Kang Department of Anatomy, Physiology and Pharmacology, College of Veterinary Medicine, Auburn University, Auburn, Alabama, USA

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Introduction Zona glomerulosa (ZG), zona fasciculata (ZF) and zona reticularis (ZR) are the three major cortical zones (definitive zones) in the mammalian adrenal gland. In human fetal adrenals, there is another cortical layer in the inner

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Wendy B Bollag Charlie Norwood VA Medical Center, One Freedom Way, Augusta, GA, USA
Department of Physiology, Medical College of Georgia, Augusta University (formerly Georgia Regents University), Augusta, GA, USA

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have each been shown to play a role in regulating steroidogenesis in the adrenal cortex. This review discusses each in turn, as well as the evidence linking them to adrenal steroid hormone production. Figure 1 The reactions catalyzed by

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Maria Candida Barisson Villares Fragoso Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Guilherme Asmar Alencar Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Antonio Marcondes Lerario Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Isabelle Bourdeau Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Madson Queiroz Almeida Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Berenice Bilharinho Mendonca Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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André Lacroix Unidade de Suprarrenal, Instituto do Câncer de São Paulo ICESP, Département de Médecine, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Introduction Adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is a rare and insidious cause of Cushing's syndrome (CS), and accounts for <2% of all endogenous cases of CS ( Arnaldi et al . 2003 , Lacroix

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Ken Takao Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Katsumi Iizuka Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Yanyan Liu Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Teruaki Sakurai Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Sodai Kubota Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan
Yutaka Seino Distinguished Center for Diabetes Research, Kansai Electric Power Medical Research Institute, Kobe, Japan

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Saki Kubota-Okamoto Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan
Yutaka Seino Distinguished Center for Diabetes Research, Kansai Electric Power Medical Research Institute, Kobe, Japan

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Toshinori Imaizumi Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Yoshihiro Takahashi Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Yermek Rakhat Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan
Yutaka Seino Distinguished Center for Diabetes Research, Kansai Electric Power Medical Research Institute, Kobe, Japan

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Satoko Komori Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Tokuyuki Hirose Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Kenta Nonomura Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Takehiro Kato Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Masami Mizuno Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Tetsuya Suwa Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Yukio Horikawa Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan

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Masakatsu Sone Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan

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Daisuke Yabe Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, Gifu, Japan
Yutaka Seino Distinguished Center for Diabetes Research, Kansai Electric Power Medical Research Institute, Kobe, Japan
Division of Molecular and Metabolic Medicine, Department of Physiology and Cell Biology, Kobe University Graduate School of Medicine, Kobe, Japan

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reason (e.g. reduced plasma cholesterol levels), cells compensate for the cholesterol insufficiency by upregulating genes involved in cholesterol synthesis ( Brown et al. 2018 ). Adrenal cortex regulates homeostasis of glucose, lipids and minerals

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