WT1是肿瘤抑制基因,在泌尿生殖器的发育和Wilms瘤的发生中起关键作用。Wilms瘤(WT)是一种肾脏由胚胎成分构成的发展迅速的恶性混和瘤,常累及5岁前儿童,发病率为万分之一。WT1基因有10个外显子,通过选择性剪接能编码4种蛋白类型:第5外显子编码的17个氨基酸可转录或剪去;同样,在第3与第4锌指间的赖、苏、丝氨酸(KTS)也可转录或剪去。WT1中的锌指结构提示它的主要生理功能是作为DNA结合蛋白参与生长调 WT1 is a tumor suppressor gene that plays a key role in urogenital development and Wilms’ tumorigenesis. Wilms tumor (WT) is a rapidly developing malignant mixed tumor composed of embryonic components of the kidney, often affecting children up to 5 years of age with a one-in-10 incidence. The WT1 gene has 10 exons and encodes 4 protein types by alternative splicing: 17 amino acids encoded by exon 5 can be transcribed or cloned; similarly, between the 3rd and 4th zinc fingers, Su, serine (KTS) can also be transcribed or cut off. The zinc finger structure in WT1 suggests that its main physiological function is to act as a DNA-binding protein in growth regulation