Generic placeholder image

Current Molecular Medicine


ISSN (Print): 1566-5240
ISSN (Online): 1875-5666

Insights from Animal Models on the Origins and Progression of Retinoblastoma

Author(s): Marek Pacal and Rod Bremner

Volume 6, Issue 7, 2006

Page: [759 - 781] Pages: 23

DOI: 10.2174/1566524010606070759


The RB gene was discovered 20 years ago because of its role in the childhood eye cancer retinoblastoma. However, surprisingly little progress was made in defining the role of RB protein in the retina. In the last two years, new models exploiting conditional deletion of the mouse Rb gene have altered this picture radically. These models provide insight into the first Rb function, the cell of origin of retinoblastoma, the window during which Rb acts, distinct cell-specific defenses against Rb loss, the number and type of post-Rb lesions required for transformation, why pediatric tumors exist, the controversial role of the p53 pathway in retinoblastoma, and the reason why the disease is virtually unique to humans. Two years have dramatically improved our understanding of Rb function in the tissue that gave us this important tumor suppressor.

Keywords: mouse embryo fibroblasts (MEFs), Retinal Transition Cells, Viral Oncoproteins, Germline Knock-Out Mice, Chx10-Cre Mice

© 2024 Bentham Science Publishers | Privacy Policy