Molecular mechanisms of mechanical signal sensing by motile cilia in vertebrate organogenesis

多囊肾病（Polycystic kidney disease，PKD）是一种严重的脊椎动物先天性疾病，其特征是肾小管上皮细胞异常增殖，并逐渐变平，导致整个肾脏出现大量囊肿。我们发现ktu突变体的肾上皮细胞在开始尿流之前是扁平的。细胞移植实验表明，ktu似乎以细胞自主的方式引起这种形态学变化。为了进一步了解Ktu的功能，我们鉴定了Ktu相互作用蛋白，并分析了这些蛋白的作用，特别是在斑马鱼需要运动纤毛的发育事件中。

Polycystic kidney disease (PKD) is a serious congenital disease in vertebrates, in which epithelial cells in renal tubules abnormally proliferate and become flattened in shape leading to development of numerous cysts in the entire kidney.In this study, we analyzed the medaka mutant kintoun (ktu) that develops PKD. We revealed that renal epithelial cells of ktu mutant were flattened before beginning of urine flow. Cell transplantation experiments showed that ktu seemed to cause this morphological change in a cell-autonomous manner. To further understand the function of Ktu, we identified Ktu-interacting proteins and analyzed the roles of these proteins, especially in the developmental events requiring motile cilia in zebrafish.