Aberrant G-protein coupled receptor expression in relation to adrenocortical overfunction.
The aberrant adrenal expression and function of one or several G-protein coupled receptors can lead to cell proliferation and abnormal regulation of steroidogenesis in unilateral adenomas, carcinomas or in ACTH-independent macronodular adrenal hyperplasia (AIMAH). Excess cortisol secretion leading to either sub-clinical or overt Cushing's syndrome is the most prevalent phenotype reported to date. In a few patients, aberrant regulation of androgen excess has been reported. More recently, initial studies suggest that similar mechanisms are involved in the renin-independent regulation of aldosterone secretion in primary aldosteronism. In recent years, cases of familial AIMAH have been identified, and specific aberrant hormone receptors are functional in the adrenal of affected members. The identification of aberrant receptors can offer specific pharmacological approach to prevent disease progression and control abnormal steroidogenesis; alternatively, unilateral or bilateral adrenalectomy remains the treatment of choice.
Lacroix
André
A
Division of Endocrinology, Department of Medicine, Centre de recherche du Centre hospitalier de l'Université de Montréal, Université de Montréal, Montréal, QC, Canada. andre.lacroix@umontreal.ca
Bourdeau
Isabelle
I
Lampron
Antoine
A
Mazzuco
Tânia L
TL
Tremblay
Johanne
J
Hamet
Pavel
P
D000310:Adrenal Gland Neoplasms / Q000503:physiopathology; D000311:Adrenal Glands / Q000378:metabolism; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000502:physiology; D000809:Angiotensins / Q000502:physiology; D000818:Animals; D002395:Catecholamines / Q000502:physiology; D006063:Chorionic Gonadotropin / Q000502:physiology; D003480:Cushing Syndrome / Q000378:metabolism / Q000503:physiopathology*; D005749:Gastric Inhibitory Polypeptide / Q000502:physiology; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D006929:Hyperaldosteronism / Q000503:physiopathology; D006965:Hyperplasia / Q000378:metabolism; D007986:Luteinizing Hormone / Q000502:physiology; D043562:Receptors, G-Protein-Coupled / Q000096:biosynthesis*; D012701:Serotonin / Q000502:physiology
D000809:Angiotensins; D002395:Catecholamines; D006063:Chorionic Gonadotropin; D043562:Receptors, G-Protein-Coupled; D012701:Serotonin; D005749:Gastric Inhibitory Polypeptide; D000324:Adrenocorticotropic Hormone; D007986:Luteinizing Hormone; D006854:Hydrocortisone