Camp signaling in cortisol-producing adrenal adenoma

Davide Calebiro, Guido Di Dalmazi, Kerstin Bathon, Cristina L. Ronchi, Felix Beuschlein

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

The cAMP signaling pathway is one of the major players in the regulation of growth and hormonal secretion in adrenocortical cells. Although its role in the pathogenesis of adrenocortical hyperplasia associated with Cushing's syndrome has been clarified, a clear involvement of the cAMP signaling pathway and of one of its major downstream effectors, the protein kinase A (PKA), in sporadic adrenocortical adenomas remained elusive until recently. During the last year, a report by our group and three additional independent groups showed that somatic mutations of PRKACA, the gene coding for the catalytic subunit a of PKA, are a common genetic alteration in patients with Cushing's syndrome due to adrenal adenomas, occurring in 35-65% of the patients. In vitro studies revealed that those mutations are able to disrupt the association between catalytic and regulatory subunits of PKA, leading to a cAMP-independent activity of the enzyme. Despite somatic PRKACA mutations being a common finding in patients with clinically manifest Cushing's syndrome, the pathogenesis of adrenocortical adenomas associated with subclinical hypercortisolism seems to rely on a different molecular background. In this review, the role of cAMP/PKA signaling in the regulation of adrenocortical cell function and its alterations in cortisolproducing adrenocortical adenomas will be summarized, with particular focus on recent developments.

Original languageEnglish
Pages (from-to)M99-M106
Number of pages8
JournalEuropean Journal of Endocrinology
Volume173
Issue number4
DOIs
Publication statusPublished - 1 Oct 2015

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Fingerprint

Dive into the research topics of 'Camp signaling in cortisol-producing adrenal adenoma'. Together they form a unique fingerprint.

Cite this