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Retinoblastoma in transgenic mice.

Authors
  • Windle, J J
  • Albert, D M
  • O'Brien, J M
  • Marcus, D M
  • Disteche, C M
  • Bernards, R
  • Mellon, P L
Type
Published Article
Journal
Nature
Publication Date
Feb 15, 1990
Volume
343
Issue
6259
Pages
665–669
Identifiers
PMID: 1689463
Source
Medline
License
Unknown

Abstract

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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