Abnormal expression and function of hormone receptors in adrenal Cushing's syndrome.
The initial description of GIP-dependent Cushing's syndrome suggested that abnormal or ectopic expression of adrenal receptors for various ligands may underlie other cases of ACTH-independent hypercortisolism. GIP-dependent Cushing's syndrome has been described in patients with unilateral adenomas or bilateral ACTH-independent macronodular adrenal hyperplasia (AIMAH) and results from the adrenal overexpression of non-mutated GIP receptor. In AIMAH, other patients were identified in whom regulation of cortisol production resulted from an abnormal adrenocortical response either to vasopressin, beta-adrenergic receptor agonists, hCG/LH, or serotonin 5-HT-4 receptor agonists. The identification of the presence of an abnormal adrenal receptor offers the possibility of a new pharmacological approach to control hypercortisolism by suppressing the endogenous ligands or by using specific antagonists of the abnormal receptor.
Lacroix
A
A
Department of Medicine, Research Center, Centre Hospitalier de l'Université de Montréal, Canada.
N'Diaye
N
N
Mircescu
H
H
Hamet
P
P
Tremblay
J
J
D000302:Adrenal Cortex / Q000378:metabolism*; D002395:Catecholamines / Q000502:physiology; D003480:Cushing Syndrome / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism* / Q000503:physiopathology; D005749:Gastric Inhibitory Polypeptide / Q000502:physiology; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D011956:Receptors, Cell Surface / Q000378:metabolism* / Q000502:physiology; D014667:Vasopressins / Q000494:pharmacology
D002395:Catecholamines; D011956:Receptors, Cell Surface; D014667:Vasopressins; D005749:Gastric Inhibitory Polypeptide; D006854:Hydrocortisone