Ton C C, Huff V, Call K M, Cohn S, Strong L C, Housman D E, Saunders G F
Department of Biochemistry and Molecular Biology, University of Texas M. D. Anderson Cancer Center, Houston 77030.
Genomics. 1991 May;10(1):293-7. doi: 10.1016/0888-7543(91)90516-h.
The development of Wilms tumor (WT) has been associated with the inactivation of a "tumor suppressor" locus in human chromosome 11 band p13. Several WTs that exhibit homozygous deletions of an 11p13 candidate WT gene in its entirety have been reported. We report here a partial deletion of the candidate gene which, upon comparison with other documented homozygous deletions, permitted a precise definition of the critical genomic target in Wilms tumor. The smallest region of overlap between these deletions is a 16-kb segment of DNA encompassing the 5' exon(s) of an 11p13 gene coding for a zinc finger protein, together with an associated CpG island. This finding supports the notion that the candidate gene in question corresponds to the 11p13 WT1 Wilms tumor locus.
肾母细胞瘤（WT）的发生与人类11号染色体p13带中一个“肿瘤抑制”基因座的失活有关。已有报道称，一些WT患者的11p13候选WT基因完全发生了纯合缺失。我们在此报告该候选基因的部分缺失情况，通过与其他已记录的纯合缺失进行比较，得以精确界定肾母细胞瘤中的关键基因组靶点。这些缺失之间最小的重叠区域是一段16 kb的DNA片段，它包含一个编码锌指蛋白的11p13基因的5'外显子以及一个相关的CpG岛。这一发现支持了以下观点，即所讨论的候选基因对应于11p13 WT1肾母细胞瘤基因座。
