Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant.
Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant.
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DOI：
10.1111/j.1523-1755.2005.00378.x
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发表时间：
2005-07
影响因子：
19.6
通讯作者：
Emiko Mochizuki;K. Fukuta;T. Tada;T. Harada;Naoki Watanabe;S. Matsuo;H. Hashimoto;K. Ozato;Y. Wakamatsu
中科院分区：
文献类型：
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作者：
Emiko Mochizuki;K. Fukuta;T. Tada;T. Harada;Naoki Watanabe;S. Matsuo;H. Hashimoto;K. Ozato;Y. Wakamatsu
背景多囊肾病是一种常见的遗传性疾病。已经产生了许多鼠和斑马鱼突变体，并分别作为后肾和前肾模型用于PKD的研究。在这里，我们报告了青鳉（青鳉）的突变体，开发了许多囊肿在肾脏中的成年鱼在常染色体隐性的方式作为PKD的中肾模型。方法从形态学、组织学和超微结构特征方面描述青鳉pc突变体的表型。通过将pc突变体与来自该透明原种的鱼杂交产生pc透明原种，并用于透过活鱼的透明体壁观察肾脏。结果该突变体成年后出现双侧肾脏巨大肿大。它们在2月龄内正常性成熟，并在6月龄内死亡。受影响的肾脏被许多充满液体的囊肿占据，这些囊肿由变弱的鳞状上皮细胞排列。在显微镜下，10日龄鱼苗的前肾和20日龄鱼苗的中肾开始形成囊状结构。PC透明股票对于观察活鱼的疾病进展很有用。结论：青鳉pc突变体的肾脏疾病是PKD的中肾对应物，特别是常染色体显性PKD，基于其形态学、组织学和超微结构特征，进展缓慢。
BACKGROUND Polycystic kidney disease (PKD) is a common hereditary disease. A number of murine and zebrafish mutants have been generated and used for the study of PKD as metanephric and pronephric models, respectively. Here, we report a medaka (Oryzias latipes) mutant that develops numerous cysts in the kidney in adulthood fish in an autosomal-recessive manner as a mesonephric model of PKD. METHODS The phenotypes of the medaka pc mutant were described in terms of morphologic, histologic, and ultrastructural features. The pc see-through stock was produced by crossing a pc mutant and a fish from the see-through stock and used for observing the kidney through the transparent body wall of a live fish. RESULTS The mutant developed bilateral massive enlargement of the kidney in adulthood. They sexually matured normally within 2 months of age and died within 6 months of age. The affected kidney was occupied by numerous, fluid-filled cysts, which were lined by attenuated squamous epithelial cells. Developmentally, cystic formation began in the pronephros in 10-day-old fry and in the mesonephros in 20-day-old fry at the microscopic level. The pc see-through stock was useful in observing disease progression in live fish. CONCLUSION The kidney disorder that develops in the medaka pc mutant is a mesonephric counterpart of PKD, particularly an autosomal-dominant PKD, based on its morphologic, histologic, and ultrastructural features, and slow progression.