Defects in cortisol metabolism causing low-renin hypertension

Endocr Res. 1991;17(1-2):85-107. doi: 10.1080/07435809109027191.

Abstract

Deficiency of steroid 11 beta-hydroxylase, which is a mitochondrial cytochrome P450 required for cortisol and aldosterone synthesis, causes hypertension as well as virilization. In addition, abnormal regulation of this enzyme or a closely related isozyme may be involved in an autosomal dominant form of inherited hypertension, dexamethasone-suppressible hyperaldosteronism. An enzyme that catalyzes the interconversion of cortisol and cortisone, 11 beta-hydroxysteroid dehydrogenase, may be defective in an autosomal recessive form of hypertension termed apparent mineralocorticoid excess. The molecular bases of these forms of hypertension will be elucidated by identifying mutations in the 11 beta-hydroxylase and 11 beta-hydroxysteroid dehydrogenase genes and expressing normal and mutagenized enzymes in cultured cells.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Amino Acid Sequence
  • Amino Acids / analysis
  • Cytochrome P-450 Enzyme System / analysis
  • Cytochrome P-450 Enzyme System / genetics
  • Cytochrome P-450 Enzyme System / metabolism
  • Humans
  • Hydrocortisone / metabolism*
  • Hypertension / metabolism*
  • Molecular Sequence Data
  • Renin / metabolism*
  • Steroid 11-beta-Hydroxylase / analysis
  • Steroid 11-beta-Hydroxylase / genetics
  • Steroid 11-beta-Hydroxylase / metabolism

Substances

  • Amino Acids
  • Cytochrome P-450 Enzyme System
  • Steroid 11-beta-Hydroxylase
  • Renin
  • Hydrocortisone