The Potential Role of Aldosterone-Producing Cell Clusters in Adrenal Disease
Horm Metab Res DOI: 10.1055/a-1128-0421Primary aldosteronism (PA) is the most common cause of secondary hypertension.
The hallmark of PA is adrenal production of aldosterone under suppressed renin
conditions. PA subtypes include adrenal unilateral and bilateral
hyperaldosteronism. Considerable progress has been made in defining the role for
somatic gene mutations in aldosterone-producing adenomas (APA) as the primary
cause of unilateral PA. This includes the use of next-generation sequencing
(NGS) to define recurrent somatic mutations in APA that disrupt calcium
signaling, increase aldosterone synthase (CYP11B2) expression, and aldosterone
production. The use of CYP11B2 immunohistochemistry on adrenal glands from
normal subjects, patients with unilateral and bilateral PA has allowed the
identification of CYP11B2-positive cell foci, termed aldosterone-producing cell
clusters (APCC). APCC lie beneath the adrenal capsule and like APA, many APCC
harbor somatic gene mutations known to increase aldosterone production. These
findings suggest that APCC may play a role in pathologic progression of PA.
Herein, we provide an update on recent research directed at characterizing APCC
and also discuss the unanswered questions related to the role of APCC in PA. [...] © Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: T...
Source: Hormone and Metabolic Research - Category: Endocrinology Authors: Lim, Jung Soo Rainey, William E. Tags: Review Source Type: research