| Ectopic expression of vasopressin V1b and V2 receptors in the adrenal glands of familial ACTH-independent macronodular adrenal hyperplasia. | |
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MedLine Citation:
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PMID: 16343095 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and unusual cause of Cushing's syndrome, characterized by bilateral nodular adrenocortical hyperplasia and hypersecretion of cortisol. Familial AIMAH has rarely been reported. Recently, the aberrant expression of adrenal receptors for various ligands in AIMAH patients has become important in explaining the pathogenesis of AIMAH. In this study, we present the cases of two sisters who were affected with AIMAH. PATIENTS AND MEASUREMENTS: Two sisters, aged 46 and 58, respectively, at the time of diagnosis, were found to have Cushing's syndrome due to AIMAH. We performed provocation tests with various materials and reverse transcription polymerase chain reaction (RT-PCR) with their resected adrenal tissues to examine the hyper-responsiveness to steroidogenesis and the overexpression of related receptors, respectively. RESULTS: Provocation tests in vivo indicated that arginine vasopressin (AVP) promoted cortisol secretion through vasopressin V1a as well as V1b and V2 receptors. RT-PCR analysis revealed an abnormal cDNA expression of vasopressin V1b and V2 receptors, none of which is known to be normally expressed in the adrenal glands. CONCLUSION: The expression of ectopic vasopressin V1b and V2 receptors may be involved in the aetiology of AIMAH, at least in the case of the sibling patients presented here. |
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Authors:
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Sihoon Lee; Ranjoo Hwang; Junho Lee; Yumie Rhee; Dae Jung Kim; Ung-Il Chung; Sung-Kil Lim |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Clinical endocrinology Volume: 63 ISSN: 0300-0664 ISO Abbreviation: Clin. Endocrinol. (Oxf) Publication Date: 2005 Dec |
Date Detail:
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Created Date: 2005-12-13 Completed Date: 2006-02-28 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0346653 Medline TA: Clin Endocrinol (Oxf) Country: England |
Other Details:
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Languages: eng Pagination: 625-30 Citation Subset: IM |
Affiliation:
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Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenal Glands
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metabolism*,
radiography Adrenal Hyperplasia, Congenital / genetics*, metabolism*, radiography Arginine Vasopressin / diagnostic use Female Gene Expression Humans Hydrocortisone / blood, secretion Middle Aged Pedigree RNA, Messenger / analysis Receptors, Vasopressin / genetics, metabolism* Reverse Transcriptase Polymerase Chain Reaction Tomography, X-Ray Computed |
| Chemical | |
Reg. No./Substance:
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0/RNA, Messenger; 0/Receptors, Vasopressin; 113-79-1/Arginine Vasopressin; 50-23-7/Hydrocortisone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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