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首页> 外文期刊>Neuron >Prions and Protein Assemblies that Convey Biological Information in Health and Disease
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Prions and Protein Assemblies that Convey Biological Information in Health and Disease

机译：在健康和疾病中传达生物学信息的病毒和蛋白质组装体

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Prions derived from the prion protein (PrP) were first characterized as infectious agents that transmit pathology between individuals. However, the majority of cases of neurodegeneration caused by PrP prions occur sporadically. Proteins that self-assemble as cross-beta sheet amyloids are a defining pathological feature of infectious prion disorders and all major age-associated neurodegenerative diseases. In fact, multiple non-infectious proteins exhibit properties of template-driven self-assembly that are strikingly similar to PrP. Evidence suggests that like PrP, many proteins form aggregates that propagate between cells and convert cognate monomer into ordered assemblies. We now recognize that numerous proteins assemble into macromolecular complexes as part of normal physiology, some of which are self-amplifying. This review highlights similarities among infectious and non-infectious neurodegenerative diseases associated with prions, emphasizing the normal and pathogenic roles of higher-order protein assemblies. We propose that studies of the structural and cellular biology of pathological versus physiological aggregates will be mutually informative.
机译：源自the病毒蛋白（PrP）的病毒首先被表征为在个体之间传播病理的感染因子。但是，大多数由PrP pr病毒引起的神经退行性疾病是偶发的。自组装为交叉β-折叠淀粉样蛋白的蛋白质是感染性ion病毒病和所有主要的与年龄相关的主要神经退行性疾病的病理特征。实际上，多种非感染性蛋白质表现出模板驱动的自组装特性，与PrP极为相似。有证据表明，与PrP一样，许多蛋白质形成聚集体，这些聚集体在细胞之间传播并将关联单体转化为有序的组装体。我们现在认识到，许多蛋白质作为正常生理的一部分组装成大分子复合物，其中一些是自我扩增的。这篇综述强调了与病毒相关的感染性和非感染性神经退行性疾病之间的相似性，强调了高级蛋白质组装体的正常作用和致病作用。我们建议病理性和生理性聚集体的结构和细胞生物学研究将是相互有益的。

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