Merz P A, Somerville R A, Wisniewski H M, Manuelidis L, Manuelidis E E
Nature. 1983;306(5942):474-6. doi: 10.1038/306474a0.
Scrapie of sheep, and Creutzfeldt-Jakob disease (CJD) of man belong to a group of transmissible encephalopathies which have been successfully transmitted to a variety of hosts. In susceptible hosts, these diseases are characterized by progressive degeneration of the central nervous system leading inevitably to death. The agents responsible for these diseases have not yet been identified, but they exhibit similar physicochemical characteristics. Abnormal fibrils designated 'scrapie associated fibrils' (SAF) have been observed in synaptosomal preparations of scrapie infected brain. They have never been observed in various types of control animals. We report here that SAF are present in CJD brain fractions in the experimentally transmitted disease as well as in a few naturally occurring human cases of CJD. SAF are also present in spleen extracts of animals experimentally infected with scrapie or CJD. This close association of SAF with these two diseases and two different tissues (brain and spleen) known to contain titres of infectivity, suggest that the SAF are: (1) a unique pathological response to the disease or (2) the infectious agent of these diseases.
绵羊瘙痒症和人类克雅氏病（CJD）属于一组可传播性脑病，它们已成功传播给多种宿主。在易感宿主中，这些疾病的特征是中枢神经系统进行性退化，最终不可避免地导致死亡。引发这些疾病的病原体尚未确定，但它们表现出相似的物理化学特性。在瘙痒症感染大脑的突触体制剂中观察到了被称为“瘙痒症相关纤维”（SAF）的异常纤维。在各类对照动物中从未观察到过这种纤维。我们在此报告，在实验性传播疾病的CJD脑部分以及少数自然发生的人类CJD病例中均存在SAF。SAF也存在于实验性感染瘙痒症或CJD的动物脾脏提取物中。SAF与这两种疾病以及已知含有感染滴度的两种不同组织（大脑和脾脏）密切相关，这表明SAF：（1）是对该疾病的一种独特病理反应，或者（2）是这些疾病的感染因子。
