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Horm Metab Res 2017; 49(12): 951-956
DOI: 10.1055/s-0043-122394
Review
© Georg Thieme Verlag KG Stuttgart · New York

Aldosterone-Producing Cell Clusters in Normal and Pathological States

Kei Omata
1   Department of Pathology, University of Michigan, Ann Arbor, MI, USA
2   Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University, Aoba-ku, Sendai, Miyagi, Japan
3   Division of Clinical Hypertension, Endocrinology & Metabolism, Tohoku University, Aoba-ku, Sendai, Miyagi, Japan
,
Scott A. Tomlins
1   Department of Pathology, University of Michigan, Ann Arbor, MI, USA
4   Michigan Center for Translational Pathology, University of Michigan, Ann Arbor, MI, USA
5   Department of Urology, University of Michigan, Ann Arbor, MI, USA
6   Comprehensive Cancer Center, University of Michigan, Ann Arbor, MI, USA
,
William E. Rainey
7   Department of Molecular and Integrative Physiology, University of Michigan, Ann Arbor, MI, USA
8   Department of Medicine, University of Michigan, Ann Arbor, MI, USA
› Author Affiliations
Further Information

Publication History

received 28 August 2017

accepted 30 October 2017

Publication Date:
04 December 2017 (online)

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Abstract

Primary aldosteronism (PA) significantly increases the risk of cardiovascular complications, and early diagnosis and targeted treatment based on its pathophysiology is warranted. Next-generation sequencing (NGS) has revealed recurrent somatic mutations in aldosterone-driving genes in aldosterone-producing adenoma (APA). By applying CYP11B2 (aldosterone synthase) immunohistochemistry and NGS to adrenal glands from normal subjects and PA patients, we and others have shown that CYP11B2-positive cells make small clusters, termed aldosterone-producing cell clusters (APCC), beneath the adrenal capsule, and that APCC harbor somatic mutations in genes mutated in APA. We have shown that APCC are increased in CT-negative PA adrenals, while others showed potential progression from APCC to micro APA through mutations. These results suggest that APCC are a key factor for understanding the origin of PA, and further investigation on the relation between APCC and PA is highly needed.

Key words

primary aldosteronism - aldosterone-producing cell clusters - next-generation sequencing
 

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