Retinoblastoma in transgenic mice: models of hereditary retinoblastoma.

Daniel Albert // Publications // May 01 1999

PubMed ID: 10416793

Author(s): Mills MD, Windle JJ, Albert DM. Retinoblastoma in transgenic mice: models of hereditary retinoblastoma. Surv Ophthalmol. 1999 May-Jun;43(6):508-18. Review. PMID 10416793

Journal: Survey Of Ophthalmology, Volume 43, Issue 6,

Retinoblastoma, the most common intraocular malignancy ill childhood, has served as a paradigm for the study of genetic mechanisms of oncogenesis. The retinoblastoma susceptibility gene RB1 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 occuring 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.