title: Phosphodiesterase 8B and cyclic AMP signaling in the adrenal cortex.
abstract: Bilateral adrenocortical hyperplasia (BAH) in humans and mice has been recently linked to phosphodiesterase (PDE) 8B (PDE8B) and 11 (PDE11A) defects. These findings have followed the discovery that defects of primary genes of the cyclic monophosphatase (cAMP) signaling pathway, such as guanine nucleotide binding alpha subunit and PRKAR1A, are involved in the pathogenesis of BAH in humans; complete absence of Prkar1a in the adrenal cortex of mice also led to pathology that mimicked the human disease. Here, we review the most recent findings in human and mouse studies on PDE8B, a cAMP-specific PDE that appears to be highly expressed in the adrenal cortex and whose deficiency may underlie predisposition to BAH and possibly other human diseases.
authors.affiliation: Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, 10 Center Drive, Building 10, NIH-Clinical Research Center, Room 1-3216, Bethesda, MD, 20892, USA.
authors.forename: Leticia Ferro
authors.initials: LF
authors.lastname: Leal
authors.forename: Eva
authors.initials: E
authors.lastname: Szarek
authors.forename: Fabio
authors.initials: F
authors.lastname: Faucz
authors.forename: Constantine A
authors.initials: CA
authors.lastname: Stratakis
mesh_terms: D015105:3',5'-Cyclic-AMP Phosphodiesterases / Q000172:deficiency / Q000378:metabolism*; D000302:Adrenal Cortex / Q000378:metabolism* / Q000473:pathology; D000818:Animals; D000242:Cyclic AMP / Q000378:metabolism*; D054751:Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / Q000378:metabolism*; D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / Q000378:metabolism*; D006801:Humans; D051379:Mice
chemical_list: D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; C517834:PRKAR1A protein, human; D000242:Cyclic AMP; D054751:Cyclic AMP-Dependent Protein Kinase Catalytic Subunits; C517757:PRKACA protein, human; D015105:3',5'-Cyclic-AMP Phosphodiesterases; C468781:PDE8B protein, human