title: Aldosterone secretion: a molecular perspective.
abstract: The major mineralocorticoid hormone aldosterone is secreted from the zona glomerulosa of the adrenal cortex. Aldosterone is synthesized from cholesterol via a series of hydroxylations and oxidations. The enzymes involved in these reactions are mostly members of the cytochrome P450 superfamily. The final steps of this pathway, the conversion of 11-deoxycorticosterone (DOC) to aldosterone, require conversion via the intermediates 18-hydroxy-DOC or corticosterone and 18-hydroxycorticosterone. There are significant differences between species in the number of genes that encode the P450(11beta)-related enzymes (CYP11B) involved in these steps and the zonal distribution of their expression. One enzyme is capable of 11-hydroxylation, 18-hydroxylation, and 18-oxidation of DOC to aldosterone. The genetic basis of four diseases-congenital adrenal hyperplasia due to 11beta-hydroxylase deficiency, glucocorticoid-remediable aldosteronism, aldosterone synthase deficiency type I and type II-is explicable by mutations in these cytochrome P450(11beta)-related genes.
authors.affiliation: Howard Florey Institute of Experimental Physiology and Medicine, The University of Melbourne, Parkville, Victoria, Australia.
authors.forename: W C
authors.initials: WC
authors.lastname: Boon
authors.forename: J P
authors.initials: JP
authors.lastname: Coghlan
authors.forename: K M
authors.initials: KM
authors.lastname: Curnow
authors.forename: J G
authors.initials: JG
authors.lastname: McDougall