散发性和感染性人类朊病毒病。
Sporadic and Infectious Human Prion Diseases.
机构信息
National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom.
出版信息
Cold Spring Harb Perspect Med. 2017 Jan 3;7(1):a024364. doi: 10.1101/cshperspect.a024364.
Abstract
Human prion diseases are rare neurodegenerative diseases that have become the subject of public and scientific interest because of concerns about interspecies transmission and the unusual biological properties of the causal agents: prions. These diseases are unique in that they occur in sporadic, hereditary, and infectious forms that are characterized by an extended incubation period between exposure to infection and the development of clinical illness. Silent infection can be present in peripheral tissues during the incubation period, which poses a challenge to public health, especially because prions are relatively resistant to standard decontamination procedures. Despite intense research efforts, no effective treatment has been developed for human prion diseases, which remain uniformly fatal.
摘要
人类朊病毒病是罕见的神经退行性疾病，由于对种间传播和致病因子（朊病毒）异常的生物学特性的关注，已成为公众和科学界关注的焦点。这些疾病的独特之处在于，它们以散发性、遗传性和传染性形式出现，其特征是在接触感染和临床疾病发展之间存在较长的潜伏期。潜伏期期间，潜伏感染可能存在于外周组织中，这对公共卫生构成了挑战，尤其是因为朊病毒相对耐受标准消毒程序。尽管进行了大量研究，但仍未开发出针对人类朊病毒病的有效治疗方法，这些疾病仍然是致命的。
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