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
Place of publication CHEVY CHASE
Publisher ENDOCRINE SOC
Language eng
Subject 2712 Endocrinology, Diabetes and Metabolism
1303 Biochemistry
1310 Endocrinology
1308 Clinical Biochemistry
2704 Biochemistry, medical
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