Schedl A, Hastie N
Max-Delbruck Centrum fur Molekulare Medizin, Berlin Buch, Germany.
Mol Cell Endocrinol. 1998 May 25;140(1-2):65-9. doi: 10.1016/s0303-7207(98)00031-8.
Wilms' tumour is a childhood kidney cancer, and a classic example of cancer arising through disrupted development (Armstrong et al., 1992). It is one of the most common solid paediatric malignancies, affecting one in 10000 children. The genetics of Wilms' tumour is complicated, with several different genes or chromosomal regions being implicated (Armstrong et al., 1992). However, the gene we know most about is the Wilms' tumour predisposition gene, WT1 (Bickmore et al., 1992; Bruening and Pelletier, 1996). It is now clear that mutations in this gene in humans can lead to abnormalities of the kidneys and gonads, as well as to the eponymous tumour. Also, as discussed below, WT1 is essential for kidney, testis and ovary development, as revealed in knockout mice.
肾母细胞瘤是一种儿童期肾癌，是发育紊乱引发癌症的典型例子（阿姆斯特朗等人，1992年）。它是最常见的儿童实体恶性肿瘤之一，每10000名儿童中就有1人患病。肾母细胞瘤的遗传学较为复杂，有几个不同的基因或染色体区域与之相关（阿姆斯特朗等人，1992年）。然而，我们了解最多的基因是肾母细胞瘤易感基因WT1（比克莫尔等人，1992年；布鲁宁和佩尔蒂埃，1996年）。现在已经清楚，人类该基因的突变会导致肾脏和性腺异常，以及引发这种以病名命名的肿瘤。此外，如下所述，基因敲除小鼠实验表明，WT1对肾脏、睾丸和卵巢的发育至关重要。
