Pacal Marek, Bremner Rod
Toronto Western Research Institute, University Health Network, 399 Bathurst Street, Room Mc6-424, Toronto, Ontario, M5T 2S8 Canada.
Curr Mol Med. 2006 Nov;6(7):759-81. 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.
RB基因于20年前被发现，因其在儿童眼癌视网膜母细胞瘤中所起的作用。然而，在确定RB蛋白在视网膜中的作用方面，令人惊讶的是进展甚微。在过去两年中，利用小鼠Rb基因条件性缺失的新模型彻底改变了这一局面。这些模型为我们提供了关于RB的首个功能、视网膜母细胞瘤的起源细胞、RB发挥作用的窗口期、针对RB缺失的独特细胞特异性防御机制、转化所需的RB缺失后病变的数量和类型、儿童肿瘤存在的原因、p53通路在视网膜母细胞瘤中的争议性作用，以及该疾病实际上为何在人类中独一无二的原因的深入见解。两年时间极大地增进了我们对RB在为我们提供这一重要肿瘤抑制因子的组织中的功能的理解。
