title: Multiple aberrant hormone receptors in Cushing's syndrome.
abstract: The mechanisms regulating cortisol production when ACTH of pituitary origin is suppressed in primary adrenal causes of Cushing's syndrome (CS) include diverse genetic and molecular mechanisms. These can lead either to constitutive activation of the cAMP system and steroidogenesis or to its regulation exerted by the aberrant adrenal expression of several hormone receptors, particularly G-protein coupled hormone receptors (GPCR) and their ligands. Screening for aberrant expression of GPCR in bilateral macronodular adrenal hyperplasia (BMAH) and unilateral adrenal tumors of patients with overt or subclinical CS demonstrates the frequent co-expression of several receptors. Aberrant hormone receptors can also exert their activity by regulating the paracrine secretion of ACTH or other ligands for those receptors in BMAH or unilateral tumors. The aberrant expression of hormone receptors is not limited to adrenal CS but can be implicated in other endocrine tumors including primary aldosteronism and Cushing's disease. Targeted therapies to block the aberrant receptors or their ligands could become useful in the future.
authors.affiliation: Division of EndocrinologyDepartment of Medicine, Centre de recherche du CHUM (CRCHUM), Université de Montréal, 900, Rue Saint-Denis, Room R08-474, Montréal, Québec H2X 0A9, Canada.
authors.forename: Nada
authors.initials: N
authors.lastname: El Ghorayeb
authors.affiliation: Division of EndocrinologyDepartment of Medicine, Centre de recherche du CHUM (CRCHUM), Université de Montréal, 900, Rue Saint-Denis, Room R08-474, Montréal, Québec H2X 0A9, Canada.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Bourdeau
authors.affiliation: Division of EndocrinologyDepartment of Medicine, Centre de recherche du CHUM (CRCHUM), Université de Montréal, 900, Rue Saint-Denis, Room R08-474, Montréal, Québec H2X 0A9, Canada.
authors.forename: André
authors.initials: A
authors.lastname: Lacroix
mesh_terms: D000236:Adenoma / Q000378:metabolism*; D000310:Adrenal Gland Neoplasms / Q000378:metabolism*; D003480:Cushing Syndrome / Q000378:metabolism*; D000242:Cyclic AMP / Q000378:metabolism; D015870:Gene Expression; D006801:Humans; D044103:Receptor, Melanocortin, Type 2 / Q000378:metabolism; D011943:Receptors, Adrenergic, beta / Q000378:metabolism; D043562:Receptors, G-Protein-Coupled / Q000378:metabolism*; D011964:Receptors, Gastrointestinal Hormone / Q000378:metabolism; D018027:Receptors, Glucagon / Q000378:metabolism; D011974:Receptors, LH / Q000378:metabolism; D044422:Receptors, Serotonin, 5-HT4 / Q000378:metabolism; D017483:Receptors, Vasopressin / Q000378:metabolism
chemical_list: D044103:Receptor, Melanocortin, Type 2; D011943:Receptors, Adrenergic, beta; D043562:Receptors, G-Protein-Coupled; D011964:Receptors, Gastrointestinal Hormone; D018027:Receptors, Glucagon; D011974:Receptors, LH; D017483:Receptors, Vasopressin; D044422:Receptors, Serotonin, 5-HT4; C041086:gastric inhibitory polypeptide receptor; D000242:Cyclic AMP