1Centre for Research in Endocrinology and Reproductive Sciences (CRERS), Department of Physiology and Cell Biology, University of Health Sciences (UHS), Khayaban-e-Jamia Punjab, Lahore 54600, Pakistan.
Virol J. 2011 Nov 1;8:493. doi: 10.1186/1743-422X-8-493.
Prion diseases are transmissible neurodegenerative conditions affecting human and a wide range of animal species. The pathogenesis of prion diseases is associated with the accumulation of aggregates of misfolded conformers of host-encoded cellular prion protein (PrPC). Animal prion diseases include scrapie of sheep and goats, bovine spongiform encephalopathy (BSE) or mad cow disease, transmissible mink encephalopathy, feline spongiform encephalopathy, exotic ungulate spongiform encephalopathy, chronic wasting disease of cervids and spongiform encephalopathy of primates. Although some cases of sporadic atypical scrapie and BSE have also been reported, animal prion diseases have basically occurred via the acquisition of infection from contaminated feed or via the exposure to contaminated environment. Scrapie and chronic wasting disease are naturally sustaining epidemics. The transmission of BSE to human has caused more than 200 cases of variant Cruetzfeldt-Jacob disease and has raised serious public health concerns. The present review discusses the epidemiology, clinical neuropathology, transmissibility and genetics of animal prion diseases.
朊病毒病是一种可传播的神经退行性疾病，影响人类和多种动物物种。朊病毒病的发病机制与宿主编码细胞朊蛋白（PrPC）的错误折叠构象聚集体的积累有关。动物朊病毒病包括绵羊和山羊的羊瘙痒病、牛海绵状脑病（BSE）或疯牛病、传染性水貂脑病、猫海绵状脑病、外来有蹄类海绵状脑病、鹿慢性消耗病和灵长类动物海绵状脑病。尽管也有一些散发性非典型羊瘙痒病和 BSE 的病例报告，但动物朊病毒病基本上是通过摄入受污染的饲料或接触受污染的环境而获得感染的。羊瘙痒病和慢性消耗病是自然持续流行的。BSE 传播给人类导致了 200 多例变异型克雅氏病，引起了严重的公共卫生关注。本综述讨论了动物朊病毒病的流行病学、临床神经病理学、传染性和遗传学。
