Hill Andrew F, Collinge John
Department of Neurodegenerative Diseases, Institute of Neurology, University College London, UK.
APMIS. 2002 Jan;110(1):44-53. doi: 10.1034/j.1600-0463.2002.100106.x.
Prion diseases of humans and animals are associated with the accumulation of an abnormal isoform (PrP(Sc)) of the host-encoded prion protein (PrP(C)). Transmission of these diseases between mammalian species is usually limited by a 'species barrier', which can be mediated by differences in primary sequence of the prion protein between donor and host species. Studies on species barriers usually rely on the development of clinical disease in inoculated animals as an assessment of susceptibility in a particular host. Recent studies by a number of groups have demonstrated that the absence of clinical symptoms does not necessarily exclude transmission of prion disease across a species barrier. Such results indicate that subclinical or carrier states exist in these diseases, which has public health implications regarding human exposure to BSE prions and iatrogenic transmission from apparently healthy humans. Here the issue of subclinical prion diseases is reviewed and implications are discussed.
人类和动物的朊病毒疾病与宿主编码的朊病毒蛋白（PrP(C)）的异常异构体（PrP(Sc)）的积累有关。这些疾病在哺乳动物物种之间的传播通常受到“种间屏障”的限制，种间屏障可由供体和宿主物种之间朊病毒蛋白一级序列的差异介导。关于种间屏障的研究通常依赖于接种动物临床疾病的发展，以此作为特定宿主易感性的评估。一些研究小组最近的研究表明，没有临床症状并不一定排除朊病毒疾病跨种间屏障的传播。这些结果表明，这些疾病存在亚临床或携带者状态，这对人类接触疯牛病朊病毒以及来自看似健康人类的医源性传播具有公共卫生意义。本文对亚临床朊病毒疾病问题进行了综述并讨论了其影响。
