The kd/kd mouse is a model of collapsing Glomerulopathy
The kd/kd mouse is a model of collapsing Glomerulopathy
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DOI：
10.1681/asn.2005050494
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发表时间：
2005-10-01
影响因子：
13.6
通讯作者：
Nelson, PJ
中科院分区：
文献类型：
--
作者：
Barisoni, L;Madaio, MP;Nelson, PJ
塌陷性肾小球病（CG）与足细胞表型明显紊乱有关。对kd/kd小鼠进行了微囊肾小管间质性肾炎免疫和遗传病因的研究，但很少关注其肾小球病变。由于组织学检查显示CG的经典形态学特征，因此出现了kd/kd小鼠的足细胞是否表现出CG的额外表型标准的问题。利用Tg 26小鼠作为阳性对照，在两种模型上同时进行足细胞表型的免疫组织化学分析。与Tg 26肾脏相似，kd/kd肾脏中的足细胞显示出从头细胞周期蛋白D1、Ki-67和结蛋白表达，而突触足蛋白和WT-1表达缺失。电子显微镜下可见毛细血管塌陷，广泛的足突消失，足细胞内线粒体畸形。这些结果表明，kd/kd小鼠是CG的模型，并提出了可能性，人类等效的kd易感基因可能存在于CG患者。
Collapsing glomerulopathy (CG) is associated with disorders that markedly perturb the phenotype of podocytes. The kd/kd mouse has been studied for immune and genetic causes of microcystic tubulointerstitial nephritis with little attention to its glomerular lesion. Because histologic examination revealed classic morphologic features of CG, the question arises whether podocytes in kd/kd mice exhibit additional phenotypic criteria for CG. Utilizing Tg26 mice as a positive control, immunohistochemical profiling of the podocyte phenotype was conducted simultaneously on both models. Similar to Tg26 kidneys, podocytes in kd/kd kidneys showed de novo cyclin D1, Ki-67, and desmin expression with loss of synaptopodin and WT-1 expression. Electron micrographs showed collapsed capillaries, extensive foot process effacement, and dysmorphic mitochondria in podocytes. These results indicate that the kd/kd mouse is a model of CG and raise the possibility that human equivalents of the kd susceptibility gene may exist in patients with CG.