Epithelial cells lining the ducts and tubules of the kidney nephron and collecting duct have a single non-motile cilium projecting from their surface into the lumen of the tubule. These organelles were long considered vestigial remnants left as a result of evolution from a ciliated ancestor, but we now recognize them as critical sensory antennae. In the kidney, the polycystins and fibrocystin, products of the major human polycystic kidney disease genes, localize to this organelle. The polycystins and fibrocystin, through an unknown mechanism, monitor the diameter of the kidney tubules and regulate the proliferation and differentiation of the cells lining the tubule. When the polycystins, fibrocystin or cilia themselves are defective, the cell perceives this as a pro-proliferative signal, which leads to tubule dilation and cystic disease. In addition to critical roles in preventing cyst formation in the kidney, cilia are also important in cystic and fibrotic diseases of the liver and pancreas, and ciliary defects lead to a variety of developmental abnormalities that cause structural birth defects in most organs.

中文翻译：

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纤毛在胆囊肾等疾病中。

肾肾小管和收集管内衬的上皮细胞具有单个非活动性纤毛，纤毛从其表面伸入肾小管腔。这些细胞器长期以来被认为是纤毛祖先进化而留下的残余残余物，但我们现在将它们视为关键的感觉触角。在肾脏中，主要人类多囊性肾脏疾病基因产物多囊藻毒素和纤维囊藻毒素定位于该细胞器。多囊藻毒素和纤维囊藻毒素通过未知机制监测肾小管的直径，并调节肾小管内膜细胞的增殖和分化。当多囊蛋白，纤维囊蛋白或纤毛本身有缺陷时，细胞会将此信号视为促增殖信号，从而导致肾小管扩张和囊性疾病。