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Publication : Retinoblastoma in transgenic mice.

First Author  Windle JJ Year  1990
Journal  Nature Volume  343
Issue  6259 Pages  665-9
PubMed ID  1689463 Mgi Jnum  J:10320
Mgi Id  MGI:58773 Doi  10.1038/343665a0
Citation  Windle JJ, et al. (1990) Retinoblastoma in transgenic mice. Nature 343(6259):665-9
abstractText  Retinoblastoma, a malignancy of the eye occurring in young children, has been widely studied as a model for genetic predisposition to cancer. This disease is caused by mutations in both alleles of an anti-oncogene (the retinoblastoma gene, Rb) that inactivate or eliminate the Rb encoded protein, p105Rb (refs 1 and 2). Here we report that expression of a viral oncogene, the simian virus 40 T antigen, in the retina of transgenic mice produces heritable ocular tumours with histological, ultrastructural and immunohistochemical features identical to those of human retinoblastoma. Furthermore, we demonstrate a specific association between p105Rb and T antigen in mouse retinoblastoma tumour cells. Thus, the occurrence of these tumours is in vivo evidence for oncogenesis due to the ocular-specific expression of an Rb-binding oncoprotein that can functionally inactivate the Rb protein. As an animal model for heritable retinoblastoma, these mice should allow the study of the ontogeny, pathogenesis and treatment of this malignant disease.
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