Medrano Silvia, Sequeira-Lopez Maria Luisa S, Gomez R Ariel
Department of Pediatrics, School of Medicine, University of Virginia, Charlottesville, Virginia.
Department of Pediatrics, School of Medicine, University of Virginia, Charlottesville, Virginia.
Am J Pathol. 2014 Dec;184(12):3226-38. doi: 10.1016/j.ajpath.2014.08.012. Epub 2014 Oct 7.
Obstructive nephropathy, the leading cause of kidney failure in children, can be anatomic or functional. The underlying causes of functional hydronephrosis are not well understood. miRNAs, which are small noncoding RNAs, regulate gene expression at the post-transcriptional level. We found that miR-145-5p, a member of the miR-143/145 cluster that is highly expressed in smooth muscle cells of the renal vasculature, was present in the pelvicalyceal system and the ureter. To evaluate whether the miR-143/145 cluster is involved in urinary tract function we performed morphologic, functional, and gene expression studies in mice carrying a whole-body deletion of miR-143/145. miR-143/145-deficient mice developed hydronephrosis, characterized by severe papillary atrophy and dilatation of the pelvicalyceal system without obvious physical obstruction. Moreover, mutant mice showed abnormal ureteral peristalsis. The number of ureter contractions was significantly higher in miR-143/145-deficient mice. Peristalsis was replaced by incomplete, short, and more frequent contractions that failed to completely propagate in a proximal-distal direction. Microarray analysis showed 108 differentially expressed genes in ureters of miR-143/145-deficient mice. Ninety genes were up-regulated and 18 genes were down-regulated, including genes with potential regulatory roles in smooth muscle contraction and extracellular matrix-receptor interaction. We show that miR-143/145 are important for the normal peristalsis of the ureter and report an association between the expression of these miRNAs and hydronephrosis.
梗阻性肾病是儿童肾衰竭的主要原因,可分为解剖性或功能性。功能性肾积水的潜在病因尚不清楚。微小RNA(miRNA)是一类小的非编码RNA,在转录后水平调节基因表达。我们发现,miR-145-5p是miR-143/145簇的成员之一,在肾血管平滑肌细胞中高度表达,存在于肾盂肾盏系统和输尿管中。为了评估miR-143/145簇是否参与尿路功能,我们对全身缺失miR-143/145的小鼠进行了形态学、功能和基因表达研究。miR-143/145基因缺失的小鼠出现肾积水,其特征为严重的乳头萎缩和肾盂肾盏系统扩张,且无明显的物理梗阻。此外,突变小鼠输尿管蠕动异常。miR-143/145基因缺失的小鼠输尿管收缩次数显著增加。蠕动被不完全、短暂且更频繁的收缩所取代,这些收缩未能在近端至远端方向完全传导。基因芯片分析显示,miR-143/145基因缺失的小鼠输尿管中有108个差异表达基因。其中90个基因上调,18个基因下调,包括在平滑肌收缩和细胞外基质-受体相互作用中具有潜在调节作用的基因。我们表明,miR-143/145对输尿管的正常蠕动很重要,并报告了这些miRNA的表达与肾积水之间的关联。