Hope J, Ritchie L, Farquhar C, Somerville R, Hunter N
Institute for Animal Health, AFRC & MRC Neuropathogenesis Unit, Edinburgh, U.K.
Prog Clin Biol Res. 1989;317:659-67.
Scrapie is a CNS degenerative infection of sheep and goats, which is invariably fatal after incubation periods of several months to years. Related disorders are found naturally in man and other species. There is a impairment of protein catabolism in scrapie and related diseases which leads to the accumulation of sparingly-soluble protein deposits in brain. These protein aggregates may share with the amyloid of Alzheimer's disease (AD) some common stage in the biochemical pathways of their formation, although different proteins are affected in scrapie (the PrP protein) and AD (the A4-precursor protein). Recently, cattle with the clinical signs and brain pathology of a neurodegenerative disease have been reported, and this cattle disorder has been called bovine spongiform encephalopathy (BSE). BSE-affected brains contain abnormal forms of the bovine homologue of PrP. This provides biochemical evidence that BSE is cattle scrapie rather than cattle AD.
羊瘙痒病是绵羊和山羊中枢神经系统的一种退行性感染疾病，在经历数月至数年的潜伏期后必然会致命。在人类和其他物种中也自然存在相关疾病。在羊瘙痒病及相关疾病中存在蛋白质分解代谢受损的情况，这会导致在大脑中积累难溶性蛋白质沉积物。这些蛋白质聚集体在其形成的生化途径中可能与阿尔茨海默病（AD）的淀粉样蛋白有一些共同阶段，尽管在羊瘙痒病（朊蛋白）和AD（A4前体蛋白）中受影响的蛋白质不同。最近，有报道称牛出现了神经退行性疾病的临床症状和脑部病理变化，这种牛的疾病被称为牛海绵状脑病（BSE）。受BSE影响的大脑含有异常形式的牛朊蛋白同源物。这提供了生化证据，表明BSE是牛的羊瘙痒病而非牛的AD。
