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首页> 外文期刊>Cytoskeleton >Inv acts as a molecular anchor for Nphp3 and Nek8 in the proximal segment of primary cilia.
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Inv acts as a molecular anchor for Nphp3 and Nek8 in the proximal segment of primary cilia.

机译：Inv在原发性纤毛的近端部分充当Nphp3和Nek8的分子锚。

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摘要

A primary cilium is an antenna-like structure extending from the surface of most vertebrate cells. It is structurally divided along its vertical axis into sub-compartments that include the ciliary tip, the shaft, the ciliary necklace segment, the transitional zone and the basal body. We recently discovered that the shaft of the primary cilia has a distinct molecular compartment, termed the Inv compartment of primary cilia. Inv was discovered as a causative gene in inv mutant mice. It was later found to be responsible for the infantile type of nephronophthisis (NPHP2). Nephronophthisis (NPHP) is an autosomal recessive kidney disease. Nine causative genes have been identified, with all examined products thought to function in cilia, basal body and/or centrioles. However, their exact intra-ciliary localization and relationship have not been clear. Here, we report that products of Nphp3 and Nek8 (the mouse orthologs of the causative genes for NPHP3 and NPHP9, respectively) localize to the Inv compartment. We also show that Inv is essential for the compartmental localization of Nphp3 and Nek8, whereas localization of Inv does not require Nphp3 or Nek8. Nphp1 and Nphp4 also localize at the proximal region of the cilium, but not in Inv compartment. Our results indicate that Inv acts as an anchor for Nphp3 and Nek8 in the Inv compartment, and suggest that Inv compartment is a candidate site for intra-ciliary interaction of Inv, Nphp3 and Nek8.
机译：初级纤毛是从大多数脊椎动物细胞的表面延伸的类似天线的结构。它沿其垂直轴在结构上分为子隔室，其中包括睫状体尖端，轴，睫状体项链节，过渡区和基体。我们最近发现原发纤毛的杆身有一个独特的分子腔，称为原发纤毛的Inv腔。在inv突变小鼠中发现Inv是致病基因。后来发现它与婴幼儿型肾炎（NPHP2）有关。 Nephronophthisis（NPHP）是一种常染色体隐性肾脏疾病。已经鉴定出九种致病基因，所有被检查的产物均被认为在纤毛，基体和/或中心体中起作用。但是，它们的确切睫状内定位和关系尚不清楚。在这里，我们报告的产物Nphp3和Nek8（分别为NPHP3和NPHP9致病基因的小鼠直系同源基因）位于Inv区域。我们还表明，Inv对于Nphp3和Nek8的区室定位至关重要，而Inv的本地化不需要Nphp3或Nek8。 Nphp1和Nphp4也位于纤毛的近端区域，但不在Inv隔室中。我们的结果表明Inv充当Inv隔室中Nphp3和Nek8的锚点，并表明Inv隔室是Inv，Nphp3和Nek8纤毛内相互作用的候选位点。

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