PURPOSE To produce transgenic mice that express the SV40 T-antigen oncogene specifically in photoreceptor cells, giving rise to retinoblastoma tumors of photoreceptor cell origin; to characterize the mice with regard to transgene expression and pathology and to characterize the resulting tumors histologically.
METHODS Transgenic mice were generated that express T-antigen under the control of the murine interstitial retinol binding protein promoter.
RESULTS All mice produced developed either ocular or intracranial tumors, or both, at an early age. One line of mice was generated, and all mice of this line develop both retinal photoreceptor cell and pineal tumors by as early as 2 weeks of age. Cell lines have been established from both tumor types.
CONCLUSIONS These mice represent an animal model system for human trilateral retinoblastoma, in which retinoblastomas are accompanied by pineal tumors.