Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney
Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney
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DOI：
10.1073/pnas.93.4.1524
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发表时间：
1996-02-20
影响因子：
11.1
通讯作者：
Harris, PC
中科院分区：
文献类型：
--
作者：
Ward, CJ;Turley, H;Harris, PC
多囊肾病1是常见的遗传性常染色体显性遗传性多囊肾病的主要基因位点。我们用核糖核酸酶保护法研究了多囊肾病1在成人组织中的表达，发现其在成人组织中广泛表达，在脑中高表达，在肾脏中中等信号。用含有该分子C末端的重组蛋白的单抗检测了多囊蛋白在肾脏中的表达。在胎儿和成人肾脏中，染色仅限于上皮细胞，在发育中的肾单位中表达最明显，在成熟的小管中表达最明显，在鲍曼氏囊和近端输尿管芽中表达较少，在肾原区，在逗号形和S形的小体以及输尿管芽的远端分支中观察到可检测到的信号，相比之下，未诱导的间质和肾小球束在晚期胎儿(>；20周)和成人肾脏，皮质小管有较强的染色，Henle环和集合管有中等染色。这些结果表明，多囊蛋白的主要作用是在胚胎早期维持肾上皮细胞的分化和组织。有趣的是，多囊蛋白在mRNA水平和免疫组织化学监测下，在囊性上皮细胞中的表达较高，表明该病不是由于该蛋白的完全丧失所致。
Polycystic kidney disease 1 (PKD1) is the major locus of the common genetic disorder autosomal dominant polycystic kidney disease, We have studied PKD1 mRNA, with an RNase protection assay, and found widespread expression in adult tissue, with high levels in brain and moderate signal in kidney, Expression of the PKD1 protein, polycystin, was assessed in kidney using monoclonal antibodies to a recombinant protein containing the C terminus of the molecule, In fetal and adult kidney, staining is restricted to epithelial cells, Expression in the developing nephron is most prominent in mature tubules, with lesser staining in Bowman's capsule and the proximal ureteric bud, In the nephrogenic zone, detectable signal was observed in comma- and S-shaped bodies as well as the distal branches of the ureteric bud, By contrast, uninduced mesenchyme and glomerular tufts showed no staining, In later fetal (>20 weeks) and adult kidney, strong staining persists in cortical tubules with moderate staining detected in the loops of Henle and collecting ducts. These results suggest that polycystin's major role is in the maintenance of renal epithelial differentiation and organization from early fetal life, Interestingly, polycystin expression, monitored at the mRNA level and by immunohistochemistry, appears higher in cystic epithelia, indicating that the disease does not result from complete loss of the protein.