Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP
Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP
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DOI：
10.1128/jvi.78.23.13345-13350.2004
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发表时间：
2004-12-01
影响因子：
5.4
通讯作者：
Telling, GC
中科院分区：
文献类型：
--
作者：
Browning, SR;Mason, GL;Telling, GC
我们产生了表达鹿普鲁恩蛋白的小鼠，以产生一个模拟鹿和麋鹿慢性消耗性疾病(CWD)的转基因系统。虽然正常小鼠对CWD具有抵抗力，但这些转基因小鼠在脑内接种四种CWD分离株后230天内一致出现神经功能障碍的迹象。接种了转基因阵列纯合的转基因小鼠后，大约160天后出现了疾病。患病转基因小鼠的大脑表现出广泛的海绵状变性，含有异常的Pron蛋白和丰富的淀粉样斑块，其中许多是花斑。传播研究表明，在被分析的骡子、鹿和麋鹿中，相同的普恩病毒株会引起CWD。这些小鼠提供了一种新的可靠的工具来检测慢性萎缩性脑病的普恩病毒。
We generated mice expressing cervid prion protein to produce a transgenic system simulating chronic wasting disease (CWD) in deer and elk. While normal mice were resistant to CWD, these transgenic mice uniformly developed signs of neurological dysfunction similar to230 days following intracerebral inoculation with four CWD isolates. Inoculated transgenic mice homozygous for the transgene array developed disease after similar to160 days. The brains of sick transgenic mice exhibited widespread spongiform degeneration and contained abnormal prion protein and abundant amyloid plaques, many of which were florid plaques. Transmission studies indicated that the same prion strain caused CWD in the analyzed mule deer and elk. These mice provide a new and reliable tool for detecting CWD prions.