Yoder Bradley K, Tousson Albert, Millican Leigh, Wu John H, Bugg Charles E, Schafer James A, Balkovetz Daniel F
Department of Cell Biology, University of Alabama at Birmingham, 35294, USA.
Am J Physiol Renal Physiol. 2002 Mar;282(3):F541-52. doi: 10.1152/ajprenal.00273.2001.
Cilia are organelles that play diverse roles, from fluid movement to sensory reception. Polaris, a protein associated with cystic kidney disease in Tg737(o)(rpk) mice, functions in a ciliogenic pathway. Here, we explore the role of polaris in primary cilia on Madin-Darby canine kidney cells. The results indicate that polaris localization and solubility change dramatically during cilia formation. These changes correlate with the formation of basal bodies and large protein rafts at the apical surface of the epithelia. A cortical collecting duct cell line has been derived from mice with a mutation in the Tg737 gene. These cells do not develop normal cilia, which can be corrected by reexpression of the wild-type Tg737 gene. These data suggest that the primary cilia are important for normal renal function and/or development and that the ciliary defect may be a contributing factor to the cystic disease in Tg737(o)(rpk) mice. Further characterization of these cells will be important in elucidating the physiological role of renal cilia and in determining their relationship to cystic disease.
纤毛是一种细胞器，发挥着多种作用，从液体流动到感觉接收。北极星蛋白，一种与Tg737（o）（rpk）小鼠的多囊肾病相关的蛋白质，在纤毛生成途径中发挥作用。在这里，我们探讨北极星蛋白在Madin-Darby犬肾细胞的初级纤毛中的作用。结果表明，在纤毛形成过程中，北极星蛋白的定位和溶解性发生了显著变化。这些变化与上皮细胞顶端表面基体和大型蛋白质筏的形成相关。一种皮质集合管细胞系源自Tg737基因发生突变的小鼠。这些细胞不能发育出正常的纤毛，而通过野生型Tg737基因的重新表达可以纠正这一情况。这些数据表明，初级纤毛对正常肾功能和/或发育很重要，并且纤毛缺陷可能是Tg737（o）（rpk）小鼠多囊肾病的一个促成因素。对这些细胞的进一步表征对于阐明肾纤毛的生理作用以及确定它们与多囊肾病的关系将很重要。
