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Cilia in cystic kidney and other diseases

机译：纤毛在囊性肾脏等疾病

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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.
机译：上皮细胞衬里肾脏肾脏和收集管道的管道和小管具有从它们的表面突出到小管的内腔中的单个非运动纤毛。这些细胞器长期以来被认为是从被剥释的祖先的演变的结果留下的残留物，但我们现在将它们识别为关键的感觉天线。在肾脏，多环酮和纤维纤维中，主要人类多囊肾疾病基因的产物，本发明的本细胞素。通过未知机制，通过未知机制来监测肾小管的直径并调节衬里细胞的细胞的增殖和分化。当多络蛋白，纤维纤维或纤毛本身有缺陷时，细胞将其视为一种促型增殖信号，导致小管扩张和囊性疾病。除了在肾脏中预防囊肿形成的关键作用外，纤毛在肝脏和胰腺的囊性和纤维化疾病中也重要，睫状体缺陷导致各种发育异常导致大多数器官的结构出生缺陷。

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