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A mouse model for Carney complex.

Kurt J Griffin·Lawrence S Kirschner·Ludmila Matyakhina·Sotirios Stergiopoulos·Audrey Robinson-White·Frank Weinberg·Elise Meoli·Stefan R Bornstein·Constantine A Stratakis
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Abstract

Mice with complete inactivation of the type Ialpha regulatory subunit (RIalpha) of cyclic (c) AMP-dependent protein kinase (PKA) (coded by the Prkar1a gene) die early in embryonic life. To bypass the early embryonic lethality of Prkar1a-/- mice, we established transgenic mice carrying an antisense transgene for Prkar1a exon 2 (X2AS) under the control of a tetracycline-responsive promoter. Mice developed thyroid follicular hyperplasia and adenomas, adrenocortical hyperplasia, and other features reminiscent of PPNAD, and histiocytic and epithelial hyperplasias, lymphomas, and other mesenchymal tumors. This mouse provides a useful tool for the investigation of cAMP, RIalpha, and PKA functions and confirms Prkar1a's critical role in tumorigenesis in endocrine and other tissues.

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AnimalsCyclic AMPCyclic AMP-Dependent Protein Kinase RIalpha SubunitCyclic AMP-Dependent Protein KinasesDisease Models, AnimalHumansMiceMice, TransgenicMultiple Endocrine NeoplasiaProteinsRNA, MessengerTissue DistributionJournal Article

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Kurt J Griffin, Lawrence S Kirschner, Ludmila Matyakhina, Sotirios Stergiopoulos, Audrey Robinson-White, Frank Weinberg, Elise Meoli, Stefan R Bornstein, Constantine A Stratakis. 2004. A mouse model for Carney complex.. https://doi.org/10.1081/erc-200044145

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