Department of Animal Health, Neiker-Tecnalia, Derio, Bizkaia, Spain.
BMC Vet Res. 2010 Mar 26;6:17. doi: 10.1186/1746-6148-6-17.
Since 2002, an active surveillance program for transmissible spongiform encephalopathy in small ruminants in European Union countries allowed identification of a considerable number of atypical cases with similarities to the previously identified atypical scrapie cases termed Nor98.
Here we report molecular and neuropathological features of eight atypical/Nor98 scrapie cases detected between 2002 and 2009. Significant features of the affected sheep included: their relatively high ages (mean age 7.9 years, range between 4.3 and 12.8), their breed (all Latxa) and their PRNP genotypes (AFRQ/ALRQ, ALRR/ALRQ, AFRQ/AFRQ, AFRQ/AHQ, ALRQ/ALRH, ALRQ/ALRQ). All the sheep were confirmed as atypical scrapie by immunohistochemistry and immunoblotting. Two cases presented more PrP immunolabelling in cerebral cortex than in cerebellum.
This work indicates that atypical scrapie constitutes the most common small ruminant transmissible spongiform encephalopathy form in Latxa sheep in the Spanish Basque Country. Moreover, a new genotype (ALRQ/ALRH) was found associated to atypical scrapie.
自 2002 年以来，欧盟国家对小反刍动物传染性海绵状脑病进行了主动监测计划，发现了相当数量与先前确定的称为 Nor98 的非典型瘙痒病具有相似性的非典型病例。
在此，我们报告了 2002 年至 2009 年间检测到的 8 例非典型/Nor98 瘙痒病的分子和神经病理学特征。受影响绵羊的显著特征包括：年龄相对较大（平均年龄为 7.9 岁，范围为 4.3 至 12.8 岁）、品种（均为拉克斯）和 PRNP 基因型（AFRQ/ALRQ、ALRR/ALRQ、AFRQ/AFRQ、AFRQ/AHQ、ALRQ/ALRH、ALRQ/ALRQ）。所有绵羊均通过免疫组织化学和免疫印迹法确认为非典型瘙痒病。有 2 例大脑皮质的 PrP 免疫标记比小脑更明显。
这项工作表明，非典型瘙痒病是西班牙巴斯克地区拉克斯绵羊中最常见的小反刍动物传染性海绵状脑病形式。此外，还发现了一种与非典型瘙痒病相关的新基因型（ALRQ/ALRH）。
