Table 1.

Summary of current genetically modified mouse models of adrenal hyperplasia

Model	Gene	Promoter/Driver	Phenotype	Reference
Task1 KO	Kcnk3	Whole-body KO	Severe hyperaldosteronism. Disruption of normal zonation with ectopic expression of Cyp11b2 in the zG.	Heitzmann, et al. (2007)
Task3 KO	Kcnk9	Whole-body KO	Mild autonomous aldosterone production in adult animals. Severe hyperaldosteronism in newborn mice with autonomous corticosterone and progesterone secretion.	Guagliardo, et al. (2012), Bandulik, et al (2013)
Task1; Task3 KO	Kcnk3/Kcnk9	Whole-body KO	Severe hyperaldosteronism. No zonation defect.	Davies, et al. (2008)
Prkar1a2Δ/+	Prkar1a	EIIA-Cre (ubiquitous)	Spectrum of tumors highly overlapping with human CC. No adrenal phenotype.	Kirschner, et al. (2005)
rTA/X2AS	Prkar1a	Tet-Off system	Spectrum of tumors highly overlapping with human CC. Persistence of the X-zone in males and females. Nodular cortical changes. Autonomous corticosterone secretion.	Griffin, et al. (2004)
AdKO	Prkar1a	0.5 Akr1b7-Cre	Autonomous corticosterone secretion. Expansion of X-zone derived aberrant progenitor-like cells that ultimately occupy the entire cortex.	Sahut-Barnola, et al. (2010)
Men1+/−	Men1	Whole-body KO	Spectrum of tumors highly overlapping with human MEN1. Adrenal hyperplasia to adenoma to carcinoma evolution.	Crabtree, et al. (2001), Bertolino, et al. (2003), Loffler, et al. (2007), Harding, et. Al (2009)

Abbreviations: zG, zona glomerulosa; CC, Carney complex; MEN1, Multiple endocrine neoplasia type 1; Prkar1a, Protein kinase cAMPdependent regulatory subunit type I alpha; Kcnk3, Potassium channel subfamily K member 3; Kcnk9, Potassium channel subfamily K member 9, beta 1; Akr1b7, aldo-keto reductase family 1, member b7.