Department of Production Animal Clinical Sciences, Faculty of Veterinary Medicine, Norwegian University of Life Sciences, Svebastadveien 112, 4325, Sandnes, Norway.
Department of Biohazard and Pathology, Norwegian Veterinary Institute, P.O. box 64, 1431, Ås, Norway.
Vet Res. 2023 Sep 29;54(1):84. doi: 10.1186/s13567-023-01220-7.
Prion diseases, including chronic wasting disease (CWD) in cervids, are fatal neurodegenerative disorders caused by the misfolding of cellular prion proteins. CWD is known to spread among captive and free-ranging deer in North America. In 2016, an outbreak of contagious CWD was detected among wild reindeer in Norway, marking the first occurrence of the disease in Europe. Additionally, new sporadic forms of CWD have been discovered in red deer in Norway and moose in Fennoscandia. We used serial protein misfolding cyclic amplification to study the ability of Norwegian prion isolates from reindeer, red deer, and moose (two isolates), as well as experimental classical scrapie from sheep, to convert a panel of 16 brain homogenates (substrates) from six different species with various prion protein genotypes. The reindeer CWD isolate successfully converted substrates from all species except goats. The red deer isolate failed to convert sheep and goat substrates but exhibited amplification in all cervid substrates. The two moose isolates demonstrated lower conversion efficacies. The wild type isolate propagated in all moose substrates and in the wild type red deer substrate, while the other isolate only converted two of the moose substrates. The experimental classical scrapie isolate was successfully propagated in substrates from all species tested. Thus, reindeer CWD and classical sheep scrapie isolates were similarly propagated in substrates from different species, suggesting the potential for spillover of these contagious diseases. Furthermore, the roe deer substrate supported conversion of three isolates suggesting that this species may be vulnerable to prion disease.
朊病毒疾病，包括鹿科动物中的慢性消耗病（CWD），是由细胞朊病毒蛋白错误折叠引起的致命神经退行性疾病。CWD 已知在北美的圈养和自由放养的鹿群中传播。2016 年，在挪威的野生驯鹿中检测到传染性 CWD 的爆发，这标志着该疾病在欧洲的首次发生。此外，在挪威的红鹿和芬诺斯堪的亚的驼鹿中也发现了新的散发性 CWD 形式。我们使用连续蛋白质错误折叠循环扩增来研究来自驯鹿、红鹿和驼鹿（两个分离株）的挪威朊病毒分离株以及来自绵羊的实验性经典瘙痒病的能力，以转化来自六个不同物种的 16 个大脑匀浆的面板（底物）具有各种朊病毒蛋白基因型。除了山羊外，驯鹿 CWD 分离株成功地转化了所有物种的底物。红鹿分离株未能转化绵羊和山羊底物，但在所有鹿科动物底物中均表现出扩增。两个驼鹿分离株的转化效率较低。野生型分离株在所有驼鹿底物和野生型红鹿底物中繁殖，而另一个分离株仅转化了两个驼鹿底物。实验性经典瘙痒病分离株成功地在所有测试物种的底物中繁殖。因此，驯鹿 CWD 和经典绵羊瘙痒病分离株在不同物种的底物中相似地繁殖，表明这些传染性疾病可能会溢出。此外，狍子底物支持三种分离株的转化，这表明该物种可能易患朊病毒疾病。
