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Nat Rev Cancer. 2006 Feb;6(2):157-65.

Inherited disposition to cardiac myxoma development.

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Greenberg Division of Cardiology, Department of Medicine, Weill Medical College of Cornell University, 525 East 68th Street, New York, New York 10021, USA.


Carney complex is a genetic condition in which affected individuals develop benign tumours in various tissues, including the heart. Most individuals with Carney complex have a mutation in the PRKAR1A gene, which encodes the regulatory R1alpha subunit of protein kinase A - a significant component of the cyclic-AMP signalling pathway. Genetically engineered mutant Prkar1a mouse models show an increased propensity to develop tumours, and have established a role for R1alpha in initiating tumour formation and, potentially, in maintaining cell proliferation. Ongoing investigations are exploring the intersection of R1alpha-dependent cell signalling with other gene products such as perinatal myosin, mutation of which can also cause cardiac myxomas.

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