An Inherited Defect in Aldosterone Biosynthesis Caused by a Mutation in or near the Gene for Steroid 11-Hydroxylase
- 3 November 1988
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 319 (18) , 1193-1197
- https://doi.org/10.1056/nejm198811033191804
Abstract
The final step in aldosterone biosynthesis, an oxidation at position 18 of 18-hydroxycorticosterone, is catalyzed by an enzymatic activity termed corticosterone methyl oxidase II (CMO II). This activity is mediated in vitro by P450c11 (steroid 11-hydroxylase), a cytochrome P-450 enzyme that also catalyzes the preceding two steps of 11-hydroxylation and 18-hydroxylation. CMO II deficiency, an inherited defect in the 18-oxidation step, impairs aldosterone biosynthesis and thus leads to a clinical syndrome of salt wasting.This publication has 16 references indexed in Scilit:
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