In familial hyperaldosteronism type I, hybrid gene-induced aldosterone production dominates that induced by wild-type genes

Stowasser, M, Gartside, MG, Taylor, WL, Tunny, TJ and Gordon, RD (1997) In familial hyperaldosteronism type I, hybrid gene-induced aldosterone production dominates that induced by wild-type genes. Journal of Clinical Endocrinology And Metabolism, 82 11: 3670-3676. doi:10.1210/jc.82.11.3670


Author Stowasser, M
Gartside, MG
Taylor, WL
Tunny, TJ
Gordon, RD
Title In familial hyperaldosteronism type I, hybrid gene-induced aldosterone production dominates that induced by wild-type genes
Journal name Journal of Clinical Endocrinology And Metabolism   Check publisher's open access policy
ISSN 0021-972X
Publication date 1997-01-01
Year available 1997
Sub-type Article (original research)
DOI 10.1210/jc.82.11.3670
Open Access Status
Volume 82
Issue 11
Start page 3670
End page 3676
Total pages 7
Publisher ENDOCRINE SOC
Language eng
Abstract We compared the aldosterone-producing potency of the angiotensin II-sensitive wild-type aldosterone synthase genes and the ACTH-sensitive hybrid 11 beta-hydroxylase/aldosterone synthase gene by examining aldosterone, PRA, and cortisol day-curves (2-hourly levels over 24 h) in patients with familial hyperaldosteronism type I, before and during long-term (0.8-13.5 yr) glucocorticoid treatment. In 8 untreated patients, PRA levels were usually suppressed, and aldosterone correlated strongly with cortisol (r = 0.69-0.99). Fourteen studies were performed on 10 patients receiving glucocorticoid treatment that corrected hypertension, hypokalemia, and PRA suppression in all. ACTH was markedly and continuously suppressed in 6 studies, 3 of which demonstrated strong correlations between aldosterone and PRA (r = 0.77-0.92), ACTH was only partially suppressed in the remaining 8 studies; aldosterone correlated strongly: 1) with cortisol alone in 5 (r = 0.71-0.98); 2) with cortisol (r = 0.90) and PRA (r = 0.74) in one; 3) with PRA only in one (r = 0.80); and 4) with neither PRA nor cortisol in one. Unless ACTH is markedly and continuously suppressed, aldosterone is more responsive to ACTH than to renin/angiotensin II, despite the latter being unsuppressed. This is consistent with the hybrid gene being more powerfully expressed than the wild-type aldosterone synthase genes in familial hyperaldosteronism type I.
Keyword Endocrinology & Metabolism
Glucocorticoid-suppressible Hyperaldosteronism
Plasma-aldosterone
Remediable Aldosteronism
Hyper-aldosteronism
Dexamethasone
Hypertension
Cortisol
Renin
Angiotensin
Synthase
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Unknown

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
 
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Created: Tue, 14 Aug 2007, 03:02:03 EST