Trudel M, D'Agati V, Costantini F
Department of Genetics, College of Physicians and Surgeons, Columbia University, New York, New York.
Kidney Int. 1991 Apr;39(4):665-71. doi: 10.1038/ki.1991.80.
In this study, a genetic model of polycystic kidney disease (PKD) has been produced in transgenic mice bearing the murine c-myc gene driven by the SV40 enhancer and the adult beta-globin promoter. These animals reproducibly develop PKD and die of renal failure. The phenotype appears to result from the overexpression of c-myc in the renal tubular epithelium and consequent abnormal cell proliferation. These transgenic mice represent a genetic model of PKD which bears similarities to human autosomal dominant PKD (ADPKD) with respect to renal morphology, renal functional alterations and dominant transmission. Study of these transgenic mice may offer valuable insights into the pathogenesis of PKD.
在本研究中，已在携带由SV40增强子和成人β-珠蛋白启动子驱动的鼠源c-myc基因的转基因小鼠中构建了多囊肾病（PKD）的遗传模型。这些动物可重复性地发展为PKD并死于肾衰竭。该表型似乎是由于肾小管上皮中c-myc的过度表达以及随之而来的异常细胞增殖所致。这些转基因小鼠代表了一种PKD的遗传模型，在肾脏形态、肾功能改变和显性遗传方面与人类常染色体显性PKD（ADPKD）相似。对这些转基因小鼠的研究可能为PKD的发病机制提供有价值的见解。
