Distinct and common developmental expression patterns of the murine Pkd2 and Pkd1 genes
Distinct and common developmental expression patterns of the murine Pkd2 and Pkd1 genes
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DOI：
10.1016/s0925-4773(00)00257-4
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发表时间：
2000-05-01
影响因子：
2.6
通讯作者：
Trudel, M
中科院分区：
文献类型：
--
作者：
Guillaume, R;Trudel, M
常染色体显性多囊肾病（ADPKD）是最常见的遗传性肾脏疾病之一。至少有两个基因 PKD2 和 PKD2 与这种疾病的发生有关。我们的发病机制研究表明，人类和小鼠多囊肾病 (PKD) 涉及未能退出肾脏发育程序。因此，我们对 Pkd2 和 Pkd1 从桑椹胚阶段到成年期进行了详细的比较表达分析。与 Pkd1 一样，早在桑椹胚和囊胚阶段就检测到了 Pkd2 表达。与 Pkd1 类似，Pkd2 在小鼠发育过程中的所有间质和软骨组织中均表现出强表达。然而，与 Pkd1 相比，Pkd2 表达存在重大差异。首先，与 Pkd1 相比，神经嵴细胞来源的组织在所有年龄段都表现出低至不可检测的 Pkd2 表达。其次，间充质凝结期间未检测到 Pkd2 表达增加。第三，从小鼠发育到成年，肾脏中的高 Pkd2 表达主要位于皮质区域的管状上皮。 (C) 2000 Elsevier Science Ireland Ltd. 保留所有权利。
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most commonly inherited renal diseases. At least two genes, PKD2 and PKD2 are implicated in the development of this disease. Our pathogenetic studies showed that the human and murine polycystic kidney disease (PKD) involves failure to switch out of a renal developmental program. We have thus undertaken a detailed comparative expression analysis of Pkd2 and Pkd1 from the morula stage to adulthood. Pkd2 expression was detected as early as the morula and blastocyst stages as observed for Pkd1. Strong Pkd2 expression, similar to Pkd1, was displayed in all mesenchymal and cartilaginous tissues during mouse development. However major differences in Pkd2 expression in comparison to Pkd1 were identified. First, in contrast to Pkd1, the neural crest cell-derived tissues displayed a low to undetectable Pkd2 expression at all ages. Second, no increase in Pkd2 expression was detected during mesenchymal condensation. Third, high Pkd2 expression in the kidneys was localized mainly to the tubular epithelium of the cortical region from murine development to adulthood. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.