Whitechurch Benjamin C, Welton Jeremy M, Collins Steven J, Lawson Victoria A
Department of Pathology, The University of Melbourne, Parkville, VIC, 3010, Australia.
Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Parkville, VIC, Australia.
Adv Neurobiol. 2017;15:335-364. doi: 10.1007/978-3-319-57193-5_13.
Prion diseases are a group of invariably fatal and transmissible neurodegenerative disorders that are associated with the misfolding of the normal cellular prion protein, with the misfolded conformers constituting an infectious unit referred to as a "prion". Prions can spread within an affected organism by directly propagating this misfolding within and between cells and can transmit disease between animals of the same and different species. Prion diseases have a range of clinical phenotypes in humans and animals, with a principle determinant of this attributed to different conformations of the misfolded protein, referred to as prion strains. This chapter will describe the different clinical manifestations of prion diseases, the evidence that these diseases can be transmitted by an infectious protein and how the misfolding of this protein causes disease.
朊病毒疾病是一组必然致命且可传播的神经退行性疾病，与正常细胞朊病毒蛋白的错误折叠有关，错误折叠的构象体构成一种被称为“朊病毒”的感染性单位。朊病毒可通过在细胞内和细胞间直接传播这种错误折叠在受感染生物体中扩散，并可在同种和不同种动物之间传播疾病。朊病毒疾病在人类和动物中有一系列临床表型，其主要决定因素归因于错误折叠蛋白的不同构象，即朊病毒株。本章将描述朊病毒疾病的不同临床表现、这些疾病可由感染性蛋白传播的证据，以及这种蛋白的错误折叠如何导致疾病。
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