Gerlach Gary F, Wingert Rebecca A
Department of Biological Sciences and Center for Zebrafish Research, University of Notre Dame, 100 Galvin Life Sciences, Notre Dame, IN 46556, USA.
Department of Biological Sciences and Center for Zebrafish Research, University of Notre Dame, 100 Galvin Life Sciences, Notre Dame, IN 46556, USA.
Dev Biol. 2014 Dec 15;396(2):183-200. doi: 10.1016/j.ydbio.2014.08.038. Epub 2014 Oct 14.
The zebrafish pronephros provides an excellent in vivo system to study the mechanisms of vertebrate nephron development. When and how renal progenitors in the zebrafish embryo undergo tubulogenesis to form nephrons is poorly understood, but is known to involve a mesenchymal to epithelial transition (MET) and the acquisition of polarity. Here, we determined the precise timing of these events in pronephros tubulogenesis. As the ternary polarity complex is an essential regulator of epithelial cell polarity across tissues, we performed gene knockdown studies to assess the roles of the related factors atypical protein kinase C iota and zeta (prkcι, prkcζ). We found that prkcι and prkcζ serve partially redundant functions to establish pronephros tubule epithelium polarity. Further, the loss of prkcι or the combined knockdown of prkcι/ζ disrupted proximal tubule morphogenesis and podocyte migration due to cardiac defects that prevented normal fluid flow to the kidney. Surprisingly, tubule cells in prkcι/ζ morphants displayed ectopic expression of the transcription factor pax2a and the podocyte-associated genes wt1a, wt1b, and podxl, suggesting that prkcι/ζ are needed to maintain renal epithelial identity. Knockdown of genes essential for cardiac contractility and vascular flow to the kidney, such as tnnt2a, or elimination of pronephros fluid output through knockdown of the intraflagellar transport gene ift88, was not associated with ectopic pronephros gene expression, thus suggesting a unique role for prkcι/ζ in maintaining tubule epithelial identity separate from the consequence of disruptions to renal fluid flow. Interestingly, knockdown of pax2a, but not wt1a, was sufficient to rescue ectopic tubule gene expression in prkcι/ζ morphants. These data suggest a model in which the redundant activities of prkcι and prkcζ are essential to establish tubule epithelial polarity and also serve to maintain proper epithelial cell type identity in the tubule by inhibiting pax2a expression. These studies provide a valuable foundation for further analysis of MET during nephrogenesis, and have implications for understanding the pathways that affect nephron epithelial cells during kidney disease and regeneration.
斑马鱼原肾为研究脊椎动物肾单位发育机制提供了一个出色的体内系统。斑马鱼胚胎中的肾祖细胞何时以及如何经历肾小管形成以形成肾单位,目前了解甚少,但已知这涉及间充质到上皮的转变(MET)以及极性的获得。在此,我们确定了原肾肾小管形成过程中这些事件的精确时间。由于三元极性复合体是跨组织上皮细胞极性的重要调节因子,我们进行了基因敲低研究,以评估相关因子非典型蛋白激酶C iota和zeta(prkcι、prkcζ)的作用。我们发现prkcι和prkcζ在建立原肾肾小管上皮极性方面发挥部分冗余功能。此外,prkcι的缺失或prkcι/ζ的联合敲低会破坏近端肾小管形态发生和足细胞迁移,这是由于心脏缺陷阻止了正常的液体流向肾脏。令人惊讶的是,prkcι/ζ morphants中的肾小管细胞显示出转录因子pax2a以及与足细胞相关的基因wt1a、wt1b和podxl的异位表达,这表明prkcι/ζ是维持肾上皮特性所必需的。敲低对心脏收缩性和肾脏血管血流至关重要的基因,如tnnt2a,或通过敲低鞭毛内运输基因ift88消除原肾液体输出,均与原肾基因的异位表达无关,因此表明prkcι/ζ在维持肾小管上皮特性方面具有独特作用,与对肾液流破坏的后果无关。有趣的是,敲低pax2a而非wt1a足以挽救prkcι/ζ morphants中异位肾小管基因的表达。这些数据表明了一种模型,其中prkcι和prkcζ的冗余活性对于建立肾小管上皮极性至关重要,并且还通过抑制pax2a表达来维持肾小管中适当的上皮细胞类型特性。这些研究为进一步分析肾发生过程中的MET提供了有价值的基础,并对理解肾脏疾病和再生过程中影响肾单位上皮细胞的途径具有启示意义。