Prions, or infectious proteins, represent a major frontier in the study of infectious agents. The prions responsible for mammalian transmissible spongiform encephalopathies (TSEs) are due primarily to infectious self-propagation of misfolded prion proteins. TSE prion structures remain ill-defined, other than being highly structured, self-propagating, and often fibrillar protein multimers with the capacity to seed, or template, the conversion of their normal monomeric precursors into a pathogenic form. Purified TSE prions usually take the form of amyloid fibrils, which are self-seeding ultrastructures common to many serious protein misfolding diseases such as Alzheimer's, Parkinson's, Huntington's and Lou Gehrig's (amytrophic lateral sclerosis). Indeed, recent reports have now provided evidence of prion-like propagation of several misfolded proteins from cell to cell, if not from tissue to tissue or individual to individual. These findings raise concerns that various protein misfolding diseases might have spreading, prion-like etiologies that contribute to pathogenesis or prevalence.

中文翻译：

---

朊病毒和蛋白质错误折叠疾病的潜在传播能力。

朊病毒或传染性蛋白质代表了传染性病原体研究的主要前沿。负责哺乳动物传染性海绵状脑病 (TSE) 的朊病毒主要是由于错误折叠的朊病毒蛋白的传染性自我传播。TSE 朊病毒结构仍然不明确，除了高度结构化、自我传播且通常具有种子或模板能力的纤维状蛋白质多聚体，将其正常单体前体转化为致病形式。纯化的 TSE 朊病毒通常采用淀粉样原纤维的形式，这是许多严重的蛋白质错误折叠疾病如阿尔茨海默氏症、帕金森氏症、亨廷顿氏症和 Lou Gehrig 氏症（肌萎缩侧索硬化症）常见的自种超微结构。的确，最近的报告现在提供了几种错误折叠蛋白质从细胞到细胞，甚至从组织到组织或个体到个体的朊病毒样传播的证据。这些发现引起了人们的担忧，即各种蛋白质错误折叠疾病可能具有导致发病机制或流行的传播性朊病毒样病因。