Retinoblastoma in transgenic mice: Models of hereditary retinoblastoma

Retinoblastoma, the most common intraocular malignancy in childhood, has se rved as a paradigm for the study of genetic mechanisms of oncogenesis. The retinoblastoma susceptibity gene RB1 was the first tumor suppressor gene to be cloned and genetic and molecular biologic studies of this tumor have gr eatly expanded the understanding of the mechanics of tumorigenesis. Human r etinoblastoma has essentially no naturally occurring animal counterpart. Th e development of transgenic murine models of retinoblastoma have created an experimental tool for manipulation of a tumor gene system in vivo. These m odels have also enabled studies of new therapeutic modalities. This review outlines the development of the transgenic murine models of retinoblastoma, together with the genetic mechanisms of retinoblastoma origin. Current the rapeutic innovations developed by means of the transgenic models are descri bed. (Surv Ophthalmol 43:508-518, 1999. (C) 1999 by Elsevier Science Inc. A ll rights reserved.).