| First Author | Wilkes D | Year | 2006 |
| Journal | Nat Rev Cancer | Volume | 6 |
| Issue | 2 | Pages | 157-65 |
| PubMed ID | 16491075 | Mgi Jnum | J:107347 |
| Mgi Id | MGI:3620880 | Doi | 10.1038/nrc1798 |
| Citation | Wilkes D, et al. (2006) Inherited disposition to cardiac myxoma development. Nat Rev Cancer 6(2):157-65 |
| abstractText | 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. |
