Retinoblastoma in transgenic mice.

Daniel Albert // Publications // Feb 15 1990

PubMed ID: 1689463

Author(s): Windle JJ, Albert DM, O’Brien JM, Marcus DM, Disteche CM, Bernards R, Mellon PL. Retinoblastoma in transgenic mice. Nature. 1990 Feb 15;343(6259):665-9. PMID 1689463

Journal: Nature, Volume 343, Issue 6259, Feb 1990

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.