Primary generalized familial and sporadic glucocorticoid resistance (Chrousos Syndrome) and hypersensitivity

Evangelia Charmandari, Tomoshige Kino, George P. Chrousos

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The human glucocorticoid receptor (hGR) is a ubiquitously expressed intracellular, ligand-dependent transcription factor, which mediates the action of glucocorticoids and in fl uences physiologic functions essential for life. Alterations in the molecular mechanisms of hGR action modify glucocorticoid signal transduction and alter tissue sensitivity to glucocorticoids. This review summarizes the pathophysiology, molecular mechanisms, and clinical aspects of primary generalized familial and sporadic glucocorticoid resistance (PGGR or Chrousos syndrome) and hypersensitivity (PGGH). The molecular basis of Chrousos syndrome and PGGH has been ascribed to mutations in the hGR gene, which alter tissue sensitivity to glucocorticoids. The stochastic nature of glucocorticoid signaling pathways in association with the variable effect that hGR gene mutations/polymorphisms might have on glucocorticoid signal transduction indicates that alterations in hGR action may have important implications for many critical biological processes, such as the behavioral and physiologic responses to stress, intermediary metabolism, the immune and in fl ammatory reaction, as well as growth, development, and reproduction.

Original languageEnglish
Title of host publicationEndocrine Hypertension
Subtitle of host publicationUnderlying Mechanisms and Therapy
PublisherHumana Press Inc.
Pages69-87
Number of pages19
ISBN (Electronic)9781607615484
ISBN (Print)9781607615477
DOIs
Publication statusPublished - 1 Jan 2013

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Keywords

  • Glucocorticoid receptor
  • Glucocorticoid signal transduction
  • Primary generalized glucocorticoid hypersensitivity
  • Primary generalized glucocorticoid resistance

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Charmandari, E., Kino, T., & Chrousos, G. P. (2013). Primary generalized familial and sporadic glucocorticoid resistance (Chrousos Syndrome) and hypersensitivity. In Endocrine Hypertension: Underlying Mechanisms and Therapy (pp. 69-87). Humana Press Inc.. https://doi.org/10.1007/978-1-60761-548-4_4