Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium
Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium
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DOI：
10.1152/ajprenal.00273.2001
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发表时间：
2002-03-01
影响因子：
4.2
通讯作者：
Balkovetz, DF
中科院分区：
文献类型：
--
作者：
Yoder, BK;Tousson, A;Balkovetz, DF
纤毛是细胞器，发挥不同的作用，从流体运动到感觉接收。Polaris是一种与Tg 737（orpk）小鼠囊性肾病相关的蛋白质，在纤毛生成途径中发挥作用。在这里，我们探讨北极星在初级纤毛的作用，对Madin-Darby犬肾细胞。结果表明，在纤毛形成过程中，北极星的定位和溶解度发生了显著变化。这些变化与上皮细胞顶面基底体和大蛋白筏的形成有关。皮质集合管细胞系来源于Tg 737基因突变的小鼠。这些细胞不发育正常的纤毛，这可以通过野生型Tg 737基因的再表达来纠正。这些数据表明，初级纤毛是重要的正常肾功能和/或发展，纤毛缺陷可能是一个促成因素的囊性疾病Tg 737（orpk）小鼠。这些细胞的进一步表征将是重要的，在阐明肾纤毛的生理作用，并在确定它们的关系，囊性疾病。
Cilia are organelles that play diverse roles, from fluid movement to sensory reception. Polaris, a protein associated with cystic kidney disease in Tg737(orpk) 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(orpk) 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.