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pubmed-article:21047926pubmed:abstractTextCarney complex (CNC) is an autosomal dominant multiple neoplasia, caused mostly by inactivating mutations of the regulatory subunit 1A of the protein kinase A (PRKAR1A). Primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine manifestation of CNC with a great inter-individual variability. Germline, protein-truncating mutations of phosphodiesterase type 11A (PDE11A) have been described to predispose to a variety of endocrine tumors, including adrenal and testicular tumors.lld:pubmed
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pubmed-article:21047926pubmed:articleTitleFrequent phosphodiesterase 11A gene (PDE11A) defects in patients with Carney complex (CNC) caused by PRKAR1A mutations: PDE11A may contribute to adrenal and testicular tumors in CNC as a modifier of the phenotype.lld:pubmed
pubmed-article:21047926pubmed:affiliationInstitut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Paris, France.lld:pubmed
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