Chevalier G, Perbal B
Laboratoire d'oncologie virale et moléculaire, Institut Curie, Orsay, France.
Bull Cancer. 1997 Mar;84(3):289-303.
Although several cytogenetic alterations have been associated with development of Wilms' tumor, a multigenic neoplasia, molecular mechanisms of its induction, development and maintenance remain to be elucided. In order to characterize these different steps we have developed a unique animal model of Wilms' tumor constituted by the MAV-1(N) induced avian nephroblastoma. This animal model led to the discovery in our laboratory of a new gene now (nephroblastoma overexpressed gene) which is overexpressed in all avian nephroblastoma. Expression of the human nov gene (novH), which is down-regulated by WT1, is also deregulated in Wilms' tumors. Nov characteristics suggest that it would play a role in the control of cellular proliferation and differentiation. Our observations also indicate that nov could be involved in the development of Wilms' tumors, and represent a marker of their differentiation state.
尽管几种细胞遗传学改变与肾母细胞瘤（一种多基因肿瘤）的发生有关，但其诱导、发展和维持的分子机制仍有待阐明。为了表征这些不同步骤，我们建立了一种独特的肾母细胞瘤动物模型，该模型由MAV-1(N)诱导的禽类肾母细胞瘤构成。这个动物模型促使我们实验室发现了一个新基因——现在称为肾母细胞瘤过表达基因（nov），它在所有禽类肾母细胞瘤中均过表达。人nov基因（novH）的表达受WT1下调，在肾母细胞瘤中也失调。Nov的特性表明它可能在细胞增殖和分化的控制中发挥作用。我们的观察结果还表明，nov可能参与肾母细胞瘤的发展，并代表其分化状态的一个标志物。
