Sowers Levi P, Yin Terry, Mahajan Vinit B, Bassuk Alexander G
Department of Neurology, The University of Iowa , Iowa City, Iowa , USA.
J Neurogenet. 2014 Mar-Jun;28(1-2):146-52. doi: 10.3109/01677063.2014.885966. Epub 2014 Apr 7.
Motile cilia play diverse roles across phyla and cell types, and abnormalities in motile cilia lead to numerous disease states, including hydrocephalus. Although motile ciliary abnormalities in Prickle2 mutants have not yet been described, the planar cell polarity genes, including Prickle2, are implicated in the development and function of motile cilia. This report evaluates Prickle2-deficient mice for dysfunction in processes known to depend on functioning motile cilia. Prickle2-deficient mice do not develop hydrocephalus, but do display abnormal morphology and motility in the motile cilia of the ependyma. The morphology of tracheal motile cilia is also abnormal. Taken together, these results demonstrate that Prickle2 is required for normal ependymal motile cilia development and function.
运动性纤毛在不同的生物门类和细胞类型中发挥着多种作用，运动性纤毛异常会导致多种疾病状态，包括脑积水。尽管尚未描述Prickle2突变体中的运动性纤毛异常，但包括Prickle2在内的平面细胞极性基因与运动性纤毛的发育和功能有关。本报告评估了Prickle2缺陷小鼠在已知依赖于正常功能运动性纤毛的过程中的功能障碍。Prickle2缺陷小鼠不会发生脑积水，但室管膜运动性纤毛的形态和运动确实出现异常。气管运动性纤毛的形态也不正常。综上所述，这些结果表明Prickle2是正常室管膜运动性纤毛发育和功能所必需的。
