Retinoblastoma in transgenic mice: Models of hereditary retinoblastoma

Monte D. Mills, Jolene J. Windle, Daniel M. Albert

Research output: Contribution to journalReview articlepeer-review

36 Scopus citations

Abstract

Retinoblastoma, the most common intraocular malignancy in childhood, has served as a paradigm for the study of genetic mechanisms of oncogenesis. The retinoblastoma susceptibility gene RBI was the first tumor suppressor gene to be cloned, and genetic and molecular biologic studies of this tumor have greatly expanded the understanding of the mechanics of tumorigenesis. Human retinoblastoma has essentially no naturally occurring animal counterpart. The development of transgenic murine models of retinoblastoma have created an experimental tool for manipulation of a tumor gene system in vivo. These models 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 therapeutic innovations developed by means of the transgenic models are described.

Original languageEnglish (US)
Pages (from-to)508-518
Number of pages11
JournalSurvey of Ophthalmology
Volume43
Issue number6
DOIs
StatePublished - May 1999
Externally publishedYes

Keywords

  • Animal models
  • Oncogenes
  • RB1, p53
  • Retinoblastoma
  • Transgenic mice
  • Tumor suppressor genes

ASJC Scopus subject areas

  • Ophthalmology

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