Insights from animal models on the origins and progression of retinoblastoma.
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.
Pacal
Marek
M
Toronto Western Research Institute, University Health Network, 399 Bathurst Street, Room Mc6-424, Toronto, Ontario, M5T 2S8 Canada.
Bremner
Rod
R
D000818:Animals; D002471:Cell Transformation, Neoplastic; D004195:Disease Models, Animal; D018450:Disease Progression; D006801:Humans; D016160:Retinoblastoma Protein / Q000235:genetics / Q000378:metabolism*
D016160:Retinoblastoma Protein