暴露两头切维厄特 A136L141R154Q171 (AL141RQ)朊蛋白纯合基因型绵羊（PrP）大脑内的脑池准备利用英国非典型痒病的四个自然病例进行转染。直到试验最后实验动物临床表现一切正常，在7岁接种后将其宰杀。通过免疫组化而不是Western blot和酶联免疫吸附测定法观察小脑分子层发现，疾病相关的朊蛋白（朊病毒）的积累很有限。此外，朊病毒部分定位于星形胶质细胞和小胶质细胞，提示这些细胞在朊病毒加工、降解或两个过程中的作用。研究结果表明，非典型痒病传染给了AL141RQ绵羊，但这些动物作为临床无症状携带者具有较长的潜伏期。

原文摘要如下：

Transmission of atypical scrapie to homozygous ARQ sheep

Okada H¹, Miyazawa K, Imamura M, Iwamaru Y, Masujin K, Matsuura Y, Yokoyama T

Abstract：Two Cheviot ewes homozygous for the A₁₃₆L₁₄₁R₁₅₄Q₁₇₁ (AL₁₄₁RQ) prion protein (PrP) genotype were exposed intracerebrally to brain pools prepared using four field cases of atypical scrapie from the United Kingdom. Animals were clinically normal until the end of the experiment, when they were culled 7 years post-inoculation. Limited accumulation of disease-associated PrP (PrP^Sc) was observed in the cerebellar molecular layer by immunohistochemistry, but not by western blot or enzyme-linked immunosorbent assay. In addition, PrP^Sc was partially localized in astrocytes and microglia, suggesting that these cells have a role in PrP^Sc processing, degradation or both. Our results indicate that atypical scrapie is transmissible to AL₁₄₁RQ sheep, but these animals act as clinically silent carriers with long incubation times.

Keywords: prion protein，immunohistochemistry，transmission, atypical scrapie

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